BREAKING Trump Confirms Tariff-Free Beef Is Coming From Brazil And Argentina Amid Quality Controversies
Greetings, i wish to kindly address my extreme concerns with this decision to import beef from Brazil and Argentina…what about mad cow disease, or scrapie, or cjd? BREAKING Trump Confirms Tariff-Free Beef Is Coming From Brazil And Argentina Amid Quality Controversies
23h ago
BREAKING BUSINESS Trump Confirms Tariff-Free Beef Is Coming From Brazil And Argentina Amid Quality Controversies ByMary Whitfill Roeloffs,Forbes Staff.
Mary Roeloffs is a Forbes breaking news reporter covering pop culture.
Follow Author Sep 05, 2026, 10:34am EDT
TOPLINE President Donald Trump confirmed that Brazil and Argentina, meatpacking nations embroiled in quality scandals in recent months, are among the countries that will be allowed to import a combined 300,000 tons of tariff-free ground beef into the U.S. over the next three months.
US-POLITICS-TRUMP U.S. President Donald Trump in the Oval Office on Sept. 4, 2026. AFP VIA GETTY IMAGES KEY FACTS
The White House first announced its plan to bolster dwindling U.S. beef supply by waiving tariffs last month and the scheme went into effect this week, but Trump’s administration had refused to say exactly where the meat would come from. In the Oval Office Friday, Trump confirmed the meat is “coming from Argentina. It’s coming from Brazil,” as well as a “couple of other places.” Trump promised the meat is “very clean” and “very good” after American ranchers, lawmakers and experts expressed concern that the mass influx of foreign beef would flood inspection stations and introduce lower quality beef to the food supply. Trump announced the tariff-free beef plan one day after having a closed-door Oval Office meeting with Brazilian billionaire Joesley Batista, who co-owns the world’s largest meatpacking company. Brazilian beef exporters, who are usually subject to a 26.4% tariff, recently found themselves with a large backstock of beef after the European Union announced it would no longer accept imports from the country because it wasn’t meeting safety standards around the use of antibiotics.
Trump on Friday signed two executive orders about meat production: one that allows ranchers and farmers to butcher, process, package and sell their own products and another that requires country-of-origin labeling across all foreign beef. Ranchers in some states are fighting back against the rules that will make it easier for them to process their own meat, warning the current federal meat inspection process protects both public health and consumer confidence in American beef and is “NOT red tape.” Ranchers have called for the mandatory country-of-origin labeling on the lower-priced foreign beef entering the country, and are encouraging consumers to look for labels that say the product is American raised.
WHY IS BRAZILIAN AND ARGENTINE BEEF CONTROVERSIAL?
The European Union on Thursday implemented a ban on Brazilian beef imports because meat from the country isn’t meeting its standards around antibiotic use and food safety. The EU prohibits giving animals antimicrobials that are used to treat humans, including antibiotics, because overuse of can create resistant bacteria that spread to people through food, contact or the environment, making some human infections harder to treat. Argentine meat has made headlines after 30,000 pounds was allowed into Texas and Florida without being inspected by U.S. workers. China in August refused to accept a shipment of 22 tons of beef from Argentina because inspectors found evidence of chloramphenicol contamination. Chloramphenicol is an antibiotic strictly prohibited in food-producing animals under U.S. standards.
PROMOTED
HOW TO TELL IF BEEF IS FROM THE UNITED STATES
Look for stickers that say “Product of USA” or “Made in the USA.” Those mean the product comes exclusively from animals born, raised, slaughtered and processed in the United States. Other stickers to look out for will say “Born, Raised, and Harvested in the USA” or “American Grassfed Association (AGA) Certified.” If beef is imported or blended, the country of origin list will appear in small print near the nutrition label or ingredient list. Consumers can also check oval Department of Agriculture inspection stickers for an establishment number, which can be searched online to find the exact facility where the beef was processed.
KEY BACKGROUND
American ranchers are dealing with 75-year-low herd numbers after years of severe drought and water scarcity in cattle-producting states. Ground beef prices have climbed to record highs, and major meatpackers (like Tyson Foods) have begun shutting or downsizing processing plants due to a shortage of livestock. Trump’s plan will bring lean beef trimmings to be mixed with fatty American meat to make standard ground beef like 80/20 or 90/10. He has promised the meat will be sold to consumers at a 25% discount, but experts have said it’s unlikely the imports will have a significant impact on supply or prices. GOP lawmakers and representatives from big farming states have slammed the plan and said the imports will undermine American ranchers, and ranchers associations have warned about lax food safety standards in other states
https://www.forbes.com/sites/maryroeloffs/2026/09/05/trump-confirms-tariff-free-beef-is-coming-from-brazil-and-argentina-amid-quality-controversies/
re-GRAS, beef, trade talks still center stage
snip…
Where, exactly, is the beef?
The economics and politics of the cattle industry have suddenly emerged as a significant story just weeks before the midterm elections. President Trump announced last month that he would allow some 300,000 metric tons of beef to enter the U.S. under a reduced tariff rate. Trump went on to promise that the imported beef would be sold at a 25 percent discount to help reduce prices at the grocery counter. Cattle ranchers were largely caught off guard by the announcement and have raised a number of concerns. Specific details about the imports are not yet available, but the Trump administration has been issuing new directives and launching new initiatives to try to address concerns from beef industry players and Congress. For some useful context about these developments, check out our Sunday Editions that explored consolidation in the beef industry from April and the debate over mandatory country-of-origin labeling from early June. And don’t forget the screwworm outbreak that arrived in the U.S. earlier this year. Although that parasitic fly is unlikely to enter Americans’ food supply, its appearance led the U.S. government to reopen the border to imported Mexican beef in late August. This story is likely to continue to play out in a very public way as the fall election season unfolds.
https://www.foodsafetynews.com/2026/09/gras-beef-trade-talks-still-center-stage/?ref=the-sunday-edition-newsletter&attribution_id=6a9b67b473a96400016bf6f3&attribution_type=post
i’m hearing this imported beef is coming from Brazil and Argentina to the US under President Trump. this is a terrible, extremely risky idea, to try and shove Brazilian Beef down the throats of the US consumer, imho. Brazil has been caught trying to cover up mad cow disease for 2 years, cjd is out of control there, Argentina is questionable imo. this is a terrible idea on just human health risk factors alone.
i want to know where my beef comes from! i don’t want to risk eating Transmissible Spongiform Encephalopathy TSE Prion disease, neither should you, they can kill you, and Central and South America have no clue as to the BSE, Scrapie, CWD, Camel PrP TSE, Pig PrP TSE, especially Brazil…
with sad regards, terry
https://www.cattletoday.com/threads/bovine-spongiform-encephalopathy-bse-scrapie-cjd-tse-prion-in-south-america-2026-review.136659/
Bovine Spongiform Encephalopathy BSE, Scrapie, CWD, CJD, TSE prion Update September 2026
https://transmissiblespongiformencephalopathy.blogspot.com/2026/08/bovine-spongiform-encephalopathy-bse.html
https://prpsc.proboards.com/thread/238/scrapie-prion-update-september-2026
Singeltary comments and concerns…
Terry S. Singeltary Sr. Thank you for signing the Label Our Beef petition. Your signature has been recorded in support of restoring mandatory country-of-origin labeling (MCOOL) for beef. Together, we're sending a clear message to the White House and Congress: Consumers deserve to know where their beef comes from, and American cattle producers deserve the opportunity to distinguish their product in the marketplace.
Label Our Beef.
end…
Risk management strategies for bovine spongiform encephalopathy in South America
September 2010
International Journal of Risk Assessment and Management 14(3/4):254 DOI: 10.1504/IJRAM.2010.035270 Margaret A. WilsonShalu DarshanShalu DarshanDaniel KrewskiMichael G. TyshenkoMichael G. Tyshenko
Abstract and figures
Bovine spongiform encephalopathy (BSE) has never been reported in South America. As BSE began to be detected in other parts of the world, Argentina and Brazil were able to gain a larger share of the global demand for beef, with these two countries currently controlling one-third of the world export beef market. In both countries, the practice of pasturing cattle rather than feeding MBM has served to minimise the opportunity for BSE cycling, and early bans on the import of UK cattle coupled with minimal meat and bone meal (MBM) imports resulted in a very low external challenge. Specified risk material (SRM) regulations vary between countries in South America. Argentina enacted a ban on SRM in the feed chain in 2002; Brazil implemented a similar ban in early 2007. South American countries as a region had a lower risk of BSE entry, but higher cattle system instability than European Union countries.
Risk management strategies for BSE in South America
Conclusions
The implementation of preventive strategies for BSE in South America coupled with theadvantage of having a wealth of large natural grass plains used extensively for cattle grazing has played a major role in shielding the region from the influx of the disease. Argentina and especially Brazil enjoyed a boom in its beef export market as a result of BSE, with Brazil experiencing a 39% increase in beef exports in 2004 (USDA, 2005).The two countries benefited immensely from their favourable OIE and EFSA risk assessments at the time when BSE was being reported in many other countries. Other South American countries have begun to follow suit: Uruguay now exports as much beef as does Argentina, roughly 500 MT (US Department of Agriculture, 2008), and Paraguay shipped 183 MT in 2005 (Foreign Agriculture Service, USDA, 2006e).
Prior to the year 2007 the most evident difference between Argentina and Brazil in relation to BSE management was their respective policies for dealing with SRMs. Since January 2007 Brazil has implemented SRM controls. Argentina has excluded SRM from the feed chain since 2002. Brazil and Argentina recognised that there was considerable production of MBM in both respective countries intended mainly for domestic pig and poultry production; however, accidental cross-contamination could provide a means for amplification of BSE in cattle if the BSE agent were to gain entry into the national herd. This was true for Brazil prior to 2007, which, in addition to rendering SRM, imported more cattle from BSE-risk countries than did other South American countries prior to the import bans (Table 1). It must be emphasised that the predominance of pasturing in the Brazilian cattle industry and the rarity of co-farming act to minimise the risk of recycling of the infectious agent, even if it were present in imported cattle. Abetted by its willingness to provide beef raised without artificial hormones, Brazil has maintained a large share of the European beef market.
Both Argentina and Brazil were advised by EFSA to improve surveillance byincluding more at-risk animals in the testing program, such as fallen stock and emergency slaughters (European Food Safety Authority, 2005a, 2005b). The increasing orientation of the Brazilian beef industry towards the export market, prompted by a growing market share and shrinking domestic demand, makes the Brazilian industry vulnerable. The detection of a single case of BSE in the national herd could lead to catastrophic economici mpacts by limiting the beef trade in a reenactment of the situation that occurred in Canada after the detection of that country’s first BSE case in May 2003.
In the past, Argentina and Brazil have faced challenges to their beef export markets from foot and mouth disease (FMD) outbreaks. FMD outbreaks in the fall of 2005 in Brazil closed the borders of 58 markets, including Russia, Egypt, and the EU countries, to products from affected states (Meat and Livestock Australia, 2007b). Recently, the European Union banned Brazilian beef temporarily on grounds of unsatisfactory Brazilian cattle health checks for FMD (International Herald Tribune, 2008). Likewise in2001, Argentina dealt with an FMD crisis that severely challenged the beef export industry, resulting in intermittent border closures (World Organisation for Animal Health,2007b). The re-appearance of the disease after a protracted and intensive eradication program has led to a call for a ‘supranational’ surveillance system, possibly heralding broader harmonisation between countries of the region in the area of animal health (Can et al., 2004). Being mindful of the various means by which the beef export industry can be impacted negatively, regulatory bodies in these countries need to invest appropriate resources to control these emerging and reemerging cattle diseases.
(PDF) Risk management strategies for bovine spongiform encephalopathy in South America. Available from:
https://www.researchgate.net/publication/264815347_Risk_management_strategies_for_bovine_spongiform_encephalopathy_in_South_America
***> Brazil TSE Prion
Brazil 6 cases atypical BSE to date
Date: September 09, 2025 Report Number: BR2025-0024 Report Name: Livestock and Products Annual Country: Brazil Post: Brasilia Report Category: Livestock and Products Prepared By: Camila Aquino Approved By: Joseph Degreenia Report Highlights: Brazil is the second largest beef producing country and the largest beef exporting country in the world.
snip…
Animal Disease On September 9, 2024, MAPA published an Ordinance on norms of prevention and monitoring for bovine spongiform encephalopathy (BSE). Ordinance SDA/MAPA 1180/2024. To date, Brazil has only had six cases of BSE – all atypical. The country is considered as having insignificant risk for the disease per the World Organization for Animal Health (WOAH).
https://apps.fas.usda.gov/newgainapi/api/Report/DownloadReportByFileName?fileName=Livestock%20and%20Products%20Annual_Brasilia_Brazil_BR2025-0024
DIÁRIO OFICIAL DA UNIÃO Publicado em: 11/09/2024 | Edição: 176 | Seção: 1 | Página: 6 Órgão: Ministério da Agricultura e Pecuária/Secretaria de Defesa Agropecuária PORTARIA SDA/MAPA Nº 1.180, DE 9 DE SETEMBRO DE 2024 Estabelece as diretrizes do Programa Nacional de Encefalopatia Espongiforme Bovina para a aplicação de medidas oficiais de prevenção e vigilância.
https://www.gov.br/agricultura/pt-br/assuntos/insumos-agropecuarios/insumos-pecuarios/alimentacao-animal/portaria11802024.pdf
National Program for Surveillance and Prevention of Bovine Spongiform Encephalopathy - PNEEB
https://www.gov.br/agricultura/pt-br/assuntos/sustentabilidade/cesesp/eventos/vigilancia-das-sindromes-neurologicas-em-animais
Published on 06/21/2022 at 12:40 PM Updated on 06/21/2022 at 3:34 PM
https://www.gov.br/agricultura/pt-br/assuntos/sustentabilidade/cesesp/eventos/vigilancia-das-sindromes-neurologicas-em-animais/programa-nacional-de-vigilancia-e-prevencao-da-encefalopatia-espongiforme-bovina-pneeb.pdf/@@download/file
BRAZIL BSE START DATE 2023/01/18
BRAZIL BSE CONFIRMATION DATE 2023/02/22
BRAZIL BSE END DATE 2023/03/03
https://wahis.woah.org/#/in-review/4918
https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html
Brazil TSE Prion
FRIDAY, MARCH 3, 2023
Brazil MAPA OMSA Confirms BSE TSE Prion atypical H-type
https://animalhealthreportpriontse.blogspot.com/2023/03/brazil-mapa-omsa-confirms-bse-tse-prion.html
THURSDAY, FEBRUARY 23, 2023
Brazil Confirms BSE Mad Cow Case, Strain not known yet
https://animalhealthreportpriontse.blogspot.com/2023/02/brazil-confirms-bse-mad-cow-case-strain.html
TUESDAY, FEBRUARY 21, 2023
Brazil Suspected case of Bovine Spongiform Encephalopathy
https://animalhealthreportpriontse.blogspot.com/2023/02/brazil-suspected-case-of-bovine.html
''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094
FRIDAY, MARCH 17, 2023
***> Are Brazilian cervids at risk of prion diseases?
https://transmissiblespongiformencephalopathy.blogspot.com/2023/03/are-brazilian-cervids-at-risk-of-prion.html
THURSDAY, NOVEMBER 11, 2021
Brazil investigating two possible cases of mad cow disease in humans
https://creutzfeldt-jakob-disease.blogspot.com/2021/11/brazil-investigating-two-possible-cases.html
SATURDAY, NOVEMBER 13, 2021
Brazil Creutzfeldt Jakob Disease CJD TSE Prion Update 2021
https://creutzfeldt-jakob-disease.blogspot.com/2021/11/brazil-creutzfeldt-jakob-disease-cjd.html
TUESDAY, SEPTEMBER 07, 2021
Atypical Bovine Spongiform Encephalopathy BSE OIE, FDA 589.2001 FEED REGULATIONS, and Ingestion Therefrom
https://bse-atypical.blogspot.com/2021/09/atypical-bovine-spongiform.html
SATURDAY, SEPTEMBER 4, 2021
Brazil Confirms TWO More Cases of Mad Cow Disease BSE States of Mato Grosso and Minas Gerais
https://animalhealthreportpriontse.blogspot.com/2021/09/brazil-confirms-two-more-cases-of-mad.html
SATURDAY, JUNE 01, 2019
Brazil reports another cases of mad cow disease atypical BSE TSE Prion
https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html
TUESDAY, MARCH 26, 2019
Joint Statement from President Donald J. Trump USA and President Jair Bolsonaro Brazil FOREIGN POLICY BSE TSE Prion aka mad cow disease
https://bseusa.blogspot.com/2019/03/joint-statement-from-president-donald-j.html
FRIDAY, NOVEMBER 03, 2017
First case of V180I rare mutation in a Brazilian patient with Creutzfeldt-Jakob disease
http://creutzfeldt-jakob-disease.blogspot.com/2017/11/first-case-of-v180i-rare-mutation-in.html
WEDNESDAY, DECEMBER 14, 2016
English lab confirms that mad cow disease in PR Brazil was atypical 4 years ago
https://bse-atypical.blogspot.com/2016/12/english-lab-confirms-that-mad-cow.html
TUESDAY, SEPTEMBER 27, 2016
Classical Scrapie Diagnosis in ARR/ARR Sheep in Brazil
Acta Scientiae Veterinariae, 2015. 43(Suppl 1): 69.
http://scrapie-usa.blogspot.com/2016/09/classical-scrapie-diagnosis-in-arrarr.html
MONDAY, AUGUST 1, 2016
USDA Announces Reopening of Brazilian Market to U.S. Beef Exports and the Potential for Transmissible Spongiform Encephalopathy TSE prion disease
http://madcowusda.blogspot.com/2016/08/usda-announces-reopening-of-brazilian.html
Cattle in Cacoal are slaughtered after eating prohibited food Cities
Friday, December 18, 2015 - 10:21
Chicken litter
Cattle in Cacoal are slaughtered after eating prohibited food
The Agrosilvopastoral Health Defense Agency of the State of Rondônia (Idaron) in Cacoal received a complaint from a teacher at the Agricultural Family School (EFA) that three cattle from a neighboring property accidentally entered the institution and ate "chicken litter," a prohibited food under federal law. The ban is one of the main preventive measures against bovine spongiform encephalopathy (BSE), also known as mad cow disease. The animals were slaughtered last week.
Idaron inspectors collected material ingested by cattle for laboratory testing So-called "chicken litter" is the result of grinding pine sawdust (used to line the floors of farm aviaries) and poultry feces.
Normative Instruction 41/2009 of the Ministry of Agriculture, Livestock, and Supply (MAPA) prohibits feeding ruminants products containing animal proteins and fats. The Normative Instruction also provides for fines for producers who feed their herds this type of feed.
Following the complaint, Idaron's technical team collected a sample of the material consumed by the cattle and confirmed, through examination in an official MAPA laboratory, that it was "chicken litter." The animals were identified and remained on their original rural property, and their movement was prohibited.
Because it was an accident, the owner of the animals, who also notified Idaron of the incident, was not fined; he was only notified to send the cattle for slaughter. Ney Carlos Dias de Azevedo, coordinator of the EEB State Prevention and Surveillance Program, explained that mad cow disease can be transmitted to humans through the ingestion of meat from infected animals. "There is no risk of contamination through contact with the animal," he added.
The coordinator also explained that since 2012, 404 cattle have been slaughtered in Rondônia as a safety measure for having consumed feed containing animal protein, and that in 2014, 132 inspections were carried out on farms that provide supplemental feed for cattle. "Idaron is on alert. The goal is to prevent the use of prohibited foods in ruminant feed, such as meat and bone meal and chicken litter."
The disease has already caused enormous losses to cattle ranching in Europe, in addition to causing deaths from ingesting contaminated meat. In Brazil, there have been only two recorded cases of mad cow disease, which are considered an atypical form of the disease (which can occur naturally in older animals). There is no record of ingestion of prohibited food.
http://www.rondoniagora.com/noticias/bovinos+de+cacoal+sao+abatidos+apos+ingerir+alimento+proibido+2015-12-18.htm
Friday, December 18, 2015 - 10:21
Poultry litter
Cacoal cattle are slaughtered after eating forbidden food
The Health Protection Agency Agrossilvopastoril the State of Rondônia (Idaron) Cacoal received a complaint from a professor at the Family Farm School (EFA) that three cattle of a neighboring property entered accidentally in the institution and were fed "poultry litter", food prohibited, according to federal law. The ban is one of the main measures to prevent bovine spongiform encephalopathy (BSE), known as mad cow disease. The animals were slaughtered last week.
snip...see full text;
https://translate.google.com/translate?hl=en&sl=auto&tl=en&u=http%3A%2F%2Fwww.rondoniagora.com%2Fnoticias%2Fbovinos%2Bde%2Bcacoal%2Bsao%2Babatidos%2Bapos%2Bingerir%2Balimento%2Bproibido%2B2015-12-18.htm
Friday, October 30, 2015
Brazil Agriculture minister to visit Saudi Arabia over BSE mad cow trade
http://bovineprp.blogspot.com/2015/10/brazil-agriculture-minister-to-visit.html
MONDAY, MAY 5, 2014
Brazil BSE Mad Cow disease confirmed OIE 02/05/2014
http://bovineprp.blogspot.com/2014/05/brazil-bse-mad-cow-disease-confirmed.html
Monday, May 5, 2014
Brazil 2nd BSE Mad Cow disease confirmed OIE 02/05/2014
http://bovineprp.blogspot.com/2014/05/brazil-bse-mad-cow-disease-confirmed.html
Thursday, April 24, 2014
Brazil investigates possible BSE mad cow case
http://bovineprp.blogspot.com/2014/04/brazil-investigates-possible-bse-mad.html
WEDNESDAY, JANUARY 29, 2014
Another Suspect case of Creutzfeldt-Jakob disease investigated in Brazil
http://creutzfeldt-jakob-disease.blogspot.com/2014/01/another-suspect-case-of-creutzfeldt.html
THURSDAY, SEPTEMBER 26, 2013
Brazil evaluate the implementation of health rules on animal by-products and derived products SRM BSE TSE PRION aka MAD COW DISEASE
http://bse-atypical.blogspot.com/2013/09/brazil-evaluate-implementation-of.html
Wednesday, December 19, 2012
Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of Brazil
http://bse-atypical.blogspot.com/2012/12/scientific-report-of-european-food.html
***> Friday, December 07, 2012
***> ATYPICAL BSE BRAZIL 2010 FINALLY CONFIRMED OIE 2012
https://bse-atypical.blogspot.com/2012/12/atypical-bse-brazil-2010-finally.html
Updates on the WOAH activities in the field of TSEs
Natalie MOYEN Disease Status Officer
Status Department
May 14 th , 2024
Outline 1. Revised BSE standards (Terrestrial Code)
a) b) Transition process: where are we?
Official recognition of BSE risk status
i. Members/zones recognised as having a negligible or controlled BSE risk status
ii. New applications
iii. Annual reconfirmations
2. Revision of Scrapie standards (Terrestrial Code
Canada Trade Policy Monitoring Canada Bans Brazilian Beef Products 2001
https://apps.fas.usda.gov/newgainapi/api/Report/DownloadReportByFileName?fileName=Canada+Bans+Brazilian+Beef+Products_Ottawa_Canada_02-05-2001.pdf
Canada Livestock and Products Scientific Team to Assess BSE Status in Brazil 2001
https://apps.fas.usda.gov/newgainapi/api/Report/DownloadReportByFileName?fileName=Scientific+Team+to+Assess+BSE+Status+in+Brazil_Ottawa_Canada_02-14-2001.pdf
***> A critical perspective of Prion Disease surveillance in Brazil <***
2025 PRION 2025
Proceedings of the Prion 2025 - Advancing the understanding and treatment of prion diseases ISBN: 978-65-80968-48-0
A critical perspective of Prion Disease surveillance in Brazil
BARBOSA, BRENO JOSE ALENCAR PIRES 1MONTENEGRO, MARIA LUIZA VASCONCELOS 2CUNHA, JOSE ERITON GOMES DA 3 Vol 1, 2025 - 326431 Abstract Prion 2025
Abstract
Introduction: Prion Diseases (PrD) are a group of rapidly progressive dementias. Among its subtypes, Creutzfeldt-Jakob Disease (CJD) is the most common, affecting around 1 to 2 individuals per million inhabitants yearly. In 2005, Brazil's Ministry of Health (MH) initiated a surveillance program for CJD, creating a protocol to report the cases. Despite advances, the MH still struggles to make a reliable database to determine PrD profile in Brazil.
Objectives: To understand the Brazilian PrD surveillance system.
Methods: This is a retrospective and descriptive study based on the epidemiological records of CJD surveillance from 2005 to 2021 in the Ministry of Health's Epidemiological Bulletin published in 2022.
Results/Discussion: 1.576 suspected cases of CJD were reported, concentrated in the Southeast, South and Northeast regions of Brazil. Among the notifications, the following age groups predominated: 55 to 74 years (60.2%), 45 to 54 years (15%), and 75 to 85 years (11.8%). Suspected cases were mainly represented by women (53.6%), white individuals (61.4%), and residents of urban areas (90%). In this period, the expected number of cases for the Brazilian population would be 3.200.
Additionally to underreporting, the data shared by MH are limited due to the use of a database destined to mainly observe epidemic outbreaks and mismatches between official documents. Conclusion: Despite some progress since 2005, PrD surveillance in Brazil faces significant problems, due to the inaccurate treatment of these data and the lack of a specific database for CJD.
https://proceedings.science/prion-2025/papers/a-critical-perspective-of-prion-disease-surveillance-in-brazil?lang=en
SATURDAY, AUGUST 22, 2026
***> Brazil Creutzfeldt Jakob Disease CJD 547 confirmed cases in 16 years
https://creutzfeldt-jakob-disease.blogspot.com/2026/08/brazil-creutzfeldt-jakob-disease-cjd.html
https://prpsc.proboards.com/thread/233/brazil-cjd-confirmed-cases-years
***> Argentina TSE Prion
Argentina - Scrapie - Follow up report 1 GENERAL INFORMATION COUNTRY/TERRITORY OR ZONE
ZONE ANIMAL TYPE
TERRESTRIAL DISEASE CATEGORY
Listed disease EVENT ID
7429 DISEASE
Scrapie CAUSAL AGENT
Scrapie prion protein GENOTYPE / SEROTYPE / SUBTYPE
- START DATE
2025/02/20 REASON FOR NOTIFICATION
First occurrence in the country/territory DATE OF LAST OCCURRENCE
- CONFIRMATION DATE
2026/04/07 EVENT STATUS
On-going END DATE
- SELF-DECLARATION
NO REPORT INFORMATION REPORT NUMBER
Follow-up report 1 REPORT ID
FUR_183036 REPORT REFERENCE
- REPORT DATE
2026/05/12 REPORT STATUS
Validated NO EVOLUTION REPORT
https://wahis.woah.org/#/in-review/7429
WHO WAHIS WOAH Argentina - Scrapie - Immediate notification
CONFIRMATION DATE
2026/04/07 EVENT STATUS
https://wahis.woah.org/#/in-review/7429
First detection of classical scrapie in sheep imported from Paraguay in Santa Fe and Entre Ríos provinces, Argentina
MON 13 APR 2026
Source Ministry of Economy. Agriculture, Livestock, and Fisheries. National Food Safety and Quality Service (SENASA). Press release: For the first time, outbreaks of classical scrapie were detected in imported sheep. 11 Apr 2026 (in Spanish)
Key findings
On 11 Apr 2026, Argentina's National Service for Food Safety and Quality (SENASA) confirmed the country's first-ever detection of classical scrapie in three imported breeding sheep (Dorper and Santa Inés breeds) that died naturally without showing symptoms at establishments in the provinces of Santa Fe and Entre Ríos.
The affected sheep were imported from Paraguay in 2021 and 2022, underwent annual clinical inspections by SENASA veterinarians without any health issues, and were detected through active surveillance screening using ELISA testing, with confirmation by the Western Blot technique at a reference laboratory in Spain.
SENASA implemented official control and monitoring measures, including movement restrictions on affected production units, intensified surveillance, and sanitary containment protocols to prevent the spread of disease.
The detection was officially reported to the World Organisation for Animal Health (WOAH) and to Paraguay's National Service for Quality and Animal Health (SENACSA), and both countries agreed to deepen the epidemiological investigation.
Argentina is adapting its export certification to WOAH recommendations to maintain access to international markets, with safe products including embryos, hides, skins, gelatin, collagen, tallow, wool, and sheep meat with risk tissues removed. It is also proposing specific mitigation measures for live animals, protein meals, and dairy products.
Epidemiological analysis and public health impact
This detection marks a fundamental shift in Argentina's animal health status, moving the country from scrapie-free to disease-present and requiring the establishment of new surveillance infrastructure and baseline monitoring systems. Identification through active surveillance screening, rather than clinical presentation, demonstrates the effectiveness of Argentina's post-import monitoring protocols, yet also reveals that subclinical infections can persist undetected for years despite annual veterinary inspections.
The vertical transmission pattern at birth poses a significant risk of within-flock amplification, particularly because the affected animals were breeding stock imported 4–5 years ago, which could have allowed multiple lambing cycles before detection. The absence of clinical signs until natural death suggests that other subclinical carriers may exist in the affected flocks and possibly in offspring, necessitating comprehensive herd testing and assessment of genetic susceptibility.
The geographic distribution across two provinces (Santa Fe and Entre Ríos) and the common Paraguayan origin of the imports indicate a point-source introduction with the potential for wider dissemination through breeding-stock sales or animal movements before detection. The restriction measures now in place will be critical to preventing establishment in Argentina's broader sheep population, which could have substantial economic implications for the country's wool and meat export industries.
snip…
https://beaconbio.org/en/report/?search=&locations=e768e738-503e-4788-b427-dac003976695&reportid=327edbf5-7ae1-4d50-9316-43660615345c&filtersOpen=true
Outbreaks of classical scrapie were detected in imported sheep for the first time.
SENASA reported the finding in breeding specimens belonging to establishments in the provinces of Santa Fe and Entre Ríos.
April 11, 2026
The National Service for Agrifood Health and Quality (SENASA) confirmed the positive result for classical scrapie in imported breeding sheep , after the natural death without associated symptoms of three animals was recorded in establishments in Santa Fe and Entre Ríos.
The sheep involved had been imported in 2021 and 2022 from Paraguay, in accordance with the sanitary requirements and the current International Veterinary Certificate model, passing the required post-entry controls.
https://www.argentina.gob.ar/noticias/por-primera-vez-se-detectaron-brotes-de-scrapie-clasico-en-ovinos-importados
FINAL REPORT OF AN AUDIT CARRIED OUT IN ARGENTINA FROM 21 NOVEMBER 2023 TO 1 DECEMBER 2023 IN ORDER TO EVALUATE THE IMPLEMENTATION OF OFFICIAL CONTROLS ON ANIMAL BY- PRODUCTS (ABP) AND DERIVED PRODUCTS (DP)
Executive Summary
This report describes the outcome of an audit in Argentina carried out from 21 November to 1 December 2023. The audit was part of the European Commission’s Directorate-General for Health and Food Safety’s published work programme.
The objective of the audit was to assess whether the official control system over the production of rendered fat (tallow) not intended for human consumption and destined for export to the European Union (EU), as well as related certification arrangements, are sufficient to allow for reliable attestation by the responsible competent authority that the EU requirements specified in the model EU health certificate in Chapter 10(B) of Annex XV to Commission Regulation (EU) No 142/2011 are complied with. This was the first time that the Commission services have carried out an audit on the subject of animal by-products (ABP) and derived products in Argentina. The report concludes that the official control system governing the production and certification of ABP for the EU market is not fit for its intended purpose. It results in the Argentinian competent authority not being in a position to reliably verify and certify compliance of tallow with the vast majority of requirements relevant for its production and export to the EU including identifying the category of the ABP used for its manufacture, the processing method used and the marking of the product with glycerol triheptanoate.
Due to the severity and scale of the non-compliances identified during the audit, the competent authority indicated its intention to revoke the EU export authorisations of all of the tallow processing plants and delist them from TRACES-NT, pending the implementation of corrective actions.
The report contains two recommendations to the Argentinian competent authority to address the identified gaps.
snip…
Conclusions on official certification
Given the gaps in the official controls performed and the absence of certifying officers’ direct knowledge of, or evidence for the category of the raw material used for the production of tallow, the processing method used, and the use of GTH and its content, the competent authority’s attestations of compliance with most of requirements referred to in the EU health certificate are unreliable.
6 OVERALL CONCLUSION
The official control system governing the production and certification of ABP for the EU market is not fit for its intended purpose. It results in the Argentinian competent authority not being in a position to reliably verify and certify compliance of tallow with the vast majority of requirements relevant for its production and export to the EU including identifying the category of the ABP used for its manufacture, the processing method used and the marking of the product with glycerol triheptanoate.
https://ec.europa.eu/food/audits-analysis/audit-report/download/16445
1994 Sep;13(3):801-36. doi: 10.20506/rst.13.3.807.
Bovine spongiform encephalopathy surveillance in Argentina
[Article in English, Spanish] A A Schudel 1 , B J Carrillo, E J Gimeno, E L Weber, J Blanco Viera, C van Gelderen, E Ulloa, A Nader, B G Cané Affiliations expand PMID: 7949355 DOI: 10.20506/rst.13.3.807
Abstract
Bovine spongiform encephalopathy (BSE) is a new disease of cattle first described in the United Kingdom in November 1986. BSE belongs to the scrapie-related group of diseases. The epidemiological studies performed in the United Kingdom demonstrate that the BSE epidemic was caused by feeding cattle with ruminant-derived protein contaminated by a scrapie-like agent. Until June 1994, the disease had been detected in indigenous cattle in Ireland, Switzerland and France. Three cases reported in Germany, two in the Sultanate of Oman, and single cases in the Falkland Islands (Islas Malvinas), Denmark, Portugal and Canada occurred in animals imported from the United Kingdom. Several countries have implemented surveillance programmes analysing the risk factors involved in the epidemic. An analysis of risk factors conducted in Argentina shows that it is highly unlikely that BSE or scrapie exist in the country, or will arise via feed in the future. As a continuation of the analysis of risk factors, a surveillance programme was implemented in the field and in abattoirs. Specialised personnel were trained in the clinical, histopathological and biochemical detection of the disease through a network of laboratories which covered 85% of the total cattle population and 100% of the high-risk group (dairy cows over five years of age). By using a statistical procedure with reference to the bovine population in nine provinces, 1,019 brains from animals belonging to the high-risk group were selected and studied by histopathological and biochemical analyses for BSE detection. The results were negative in all cases. It can be concluded from this analysis (with a sensitivity of detection of 2.95 per 1,000, and 95% statistical confidence) that Argentina may be regarded as BSE-free, and that the importation of infected animals or by-products may represent the sole potential source of introduction of BSE infection into the country in the future.
https://pubmed.ncbi.nlm.nih.gov/7949355/
[Analysis of risk factors associated with bovine spongiform encephalopathy in Argentina]
Cané BG, Gimeno EJ, Manetti JC, Van Gelderen C, Ulloa E, Schudel AA. Rev Sci Tech. 1993 Dec;12(4):1203-34. PMID: 8312622Spanish.
https://pubmed.ncbi.nlm.nih.gov/8312622/
National MCOOL Coalition Launches Petition Calling on White House, Congress to Label Our Beef
WASHINGTON, Sept. 1, 2026
i want to know where my beef comes from! i don’t want to risk eating Transmissible Spongiform Encephalopathy TSE Prion disease, neither should you, they can kill you, and Central and South America have no clue as to the BSE, Scrapie, CWD, Camel PrP TSE, Pig PrP TSE!
https://www.cattletoday.com/threads/bovine-spongiform-encephalopathy-bse-scrapie-cjd-tse-prion-in-south-america-2026-review.136659/
***> atypical BSE and atypical Scrapie <***
Atypical BSE in cattle
THE recent diagnosis of two atypical bovine spongiform encephalopathy (BSE) cases in Great Britain (March 2023 in Cornwall and December 2024 in Dumfries and Galloway) and one in the Republic of Ireland (in November 2023) warrants a reminder about this notifiable disease.
Since 2005, a total of 17 cases have been detected in Great Britain.1 Unlike classical BSE, which resulted in over 180,000 cases in Great Britain and was predominantly associated with the consumption of feed contaminated with the BSE agent, and where the last case was confirmed in Ayrshire in May 2024, atypical BSE is believed to be a spontaneous disease in cattle found in approximately one in 1,000,000 tested cattle based on French data,2 similar to the sporadic Creutzfeldt- Jakob disease in people. There is currently no evidence that atypical BSE causes a disease in people, although it can be transmitted experimentally to other species by intracerebral inoculation, including primates.3–5 The World Organisation for Animal Health does not include atypical BSE in its geographical BSE risk status assessment.
Despite differences in terms of epidemiological, molecular and biological phenotype compared with classical BSE, atypical BSE is currently treated as if it were classical BSE in accordance with EU and UK legislation: once a case is identified, all cohort animals born and reared with the affected animal during the first 12 months of its life, and all offspring born within 24 months of its clinical onset, are culled and tested for BSE, which does seem to be at odds with the hypothesis that it is a spontaneous disease. This is more a precautionary measure to maintain confidence in the beef trade and protect consumers while more knowledge about this disease is obtained.
Almost all current knowledge on atypical BSE is based on experimental infection because this spontaneous
VET RECORD | 29 March–12 April 2025
disease has generally only been found in aged downer cows, which is difficult to replicate experimentally in the host species. Intracerebral inoculation of brain tissue from an affected cow causes disease in cattle in less than two years, unlike the natural disease that usually occurs in animals over eight years of age.
The vast majority of cases have been identified by active monitoring of fallen stock or emergency slaughter of cattle, where only the brain sample of various stages of autolysis is generally available. Little is known of where the atypical BSE agent can be found in natural disease, other than in the brain, because all the cases confirmed have been identified after death through active surveillance, by which time most peripheral tissue has been disposed of. Limited material from a single case of a naturally affected cow was tested in Italy by mouse bioassay, which found infectivity in muscle.6 In experimental disease generated by intracerebral inoculation of cattle, infectivity can be detected in the brain and spinal cord, ganglia, peripheral nerves and skeletal muscles, similar to classical BSE, but not in peripheral lymphoid tissue.6–8
Early reporting of clinical suspects is needed so that the live animal or the whole carcase can be delivered to an APHA regional laboratory for tissue sampling. This is made more difficult due to the subtlety of clinical signs based on experimental disease. Clinical cases may not be as over- reactive or nervous as classical BSE cases; some may, in fact, be dull, but what most cases have in common is that they have difficulty getting up and eventually end up as downer cows, and only the clinical history may reveal some prior behavioural or locomotor changes. High creatinine kinase serum levels and nibbling in response to scratching the tail head or back were some features in experimental disease,8, 9 but it is not known whether this is also seen in natural disease.
In general, BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment, where the blood results do not support the presence of a metabolic disease and where the cause cannot be determined with confidence.
Since BSE is a notifiable disease, suspected cases of BSE in Great Britain must be reported to the local APHA office.
Changes are imminent in the reporting of fallen stock cattle, which will require the owner to state whether the animal displayed signs of changes in behaviour, sensation or locomotion before death, in addition to the likely cause of death or disease. This is to obtain a better profile of the clinical history, if cattle are retrospectively diagnosed as BSE cases, which has happened in all BSE cases confirmed since 2010: none has been reported as a clinical suspect.
“BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment”
Timm Konold, TSE lead scientist
Brenda Rajanayagam, workgroup leader for the data systems group
APHA Weybridge, New Haw, Addlestone, Surrey KT15 3NB email: timm.konold@apha.gov.uk
Keith Meldrum, former chief veterinary officer The Orchard, Swaynes Lane, Guildford, Surrey GU1 2XX
References
1 APHA. Cattle: TSE surveillance statistics. Overview of Great Britain statistics. 2025. https://bit.ly/4ho5Nds (accessed 19 March 2025)
Atypical BSE In Cattle
https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1002/vetr.5400?campaign=woletoc
see latest on BSE TSE Prion EU;
https://bse-atypical.blogspot.com/2026/04/ireland-central-veterinary-research.html
Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle
Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *
Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.
*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca
Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.
Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.
Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.
Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.
Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.
Presentation Type: Oral Presentation
Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute
Grant Number: ALMA/APRI: 201400006, HC 414250
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE)
Component 6: Transmissible Spongiform Encephalopathies (TSEs)
Problem Statement 6A: Determine pathobiology of prion strains.
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE).
Virus and Prion Research Unit, National Animal Disease Center, Ames, Iowa
Classical BSE (C-BSE) is a prion disease of cattle that was responsible for the "mad cow disease" epizootic in Europe in the 1980s. C-BSE was determined to cause the human prion disease vCJD. Since then, atypical spontaneous strains of BSE were identified. H-BSE is one of those strains. Much research has explored the origins of C-BSE, and strain emergence from atypical H-BSE is one hypothesis. An H-BSE case was determined to have a germline mutation, an E211K substitution in the prion protein gene, which is analogous to a hereditary human prion disease. ARS scientists in Ames, Iowa reported the transmission of H-BSE from cattle, with and without the germline prion protein amino acid substitution, to cattle with various prion genotypes: EE211 (wild-type), EK211, and KK211. Results indicated a significantly shorter incubation period in K containing cattle compared to prion wild-type cattle. The scientists also explored the possibility that the C-BSE strain might have occurred after serial passages of EK211 and KK211 containing H-BSE in cattle, but results did not support this concept. This information is important to prion researchers, veterinary diagnostic laboratories, and those involved with establishing regulatory guidelines.
https://www.ars.usda.gov/ARSUserFiles/np103/AnnualReports/Final%20NP103%20FY2024%20Annual%20Report.updated%205.30.25.pdf
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research
Title: Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation
Author item Greenlee, Justin item Cassmann, Eric item MOORE, SARA JO - Oak Ridge Institute For Science And Education (ORISE) item WEST GREENLEE, HEATHER - Iowa State University
Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 6/24/2022 Publication Date: 9/16/2022 Citation: Greenlee, J.J., Cassmann, E.D., Moore, S., West Greenlee, H.M. 2022.
Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation.
Prion 2022 Conference abstracts: pushing the boundaries. 16(1):150. https://doi.org/10.1080/19336896.2022.2091286.
DOI: https://doi.org/10.1080/19336896.2022.2091286
Interpretive Summary:
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure. Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n=3) or from the US 2006 case with the E211K polymorphism (n=4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation. Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57-4.0) in the brainstem, and IHC demonstrated PrPSc throughout the brain. All cattle in the EE211 recipient group remain asymptomatic for the duration of the experiment (approximately 7 years post-inoculation).
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351
Highlights
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351
Title: A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation
Author item MOORE, S - Orise Fellow item WEST GREENLEE, M - Iowa State University item Smith, Jodi item Vrentas, Catherine item Nicholson, Eric item Greenlee, Justin
Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 8/30/2016 Publication Date: 9/15/2016 Citation: Moore, S.J., West Greenlee, M.H., Smith, J.D., Vrentas, C.E., Nicholson, E.M., Greenlee, J.J. 2016.
A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation.
Frontiers in Veterinary Science. 3:78. Interpretive
Summary: Cases of bovine spongiform encephalopathy (BSE) or mad cow disease can be subclassified into at least 3 distinct disease forms with the predominate form known as classical BSE and the others collectively referred to as atypical BSE. Atypical BSE can be further subdivided into H-type and L-type cases that are distinct from classical BSE and from each other. Both of the atypical BSE subtypes are believed to occur spontaneously, whereas classical BSE is spread through feeding contaminated meat and bone meal to cattle. Work by other research groups suggests that the stability of the distinguishing features of atypical BSE cases (phenotypical stability) can change to closely resemble classical BSE after experimental passage implicating atypical BSE as a possible origin of classical BSE. Interestingly, one case of H-type BSE in the US was associated with an inherited mutation in the prion protein gene referred to as E211K. The purpose of this work was to compare wild type and cattle with the E211K mutation after experimental inoculation with either classical BSE or H-BSE from the original E211K case. This study demonstrates that the disease features of E211K BSE-H remain stable when transmitted to cattle without the K211 polymorphism. In addition, passage of classical BSE to cattle with the K211 polymorphism results in disease with features consistent with classical BSE and not a switch to atypical BSE-H as a result of the K211 polymorphism. As the origin of classical, feedborne BSE remains unknown and low numbers of atypical BSE are diagnosed each year, parties with interest in the cattle and beef industries and regulatory officials responsible for safe feeding practices of cattle will be interested in this work.
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (BSE-H) was diagnosed in a cow that was associated with a heritable polymorphism in the bovine prion protein gene (PRNP) resulting in a lysine for glutamine amino acid substitution at codon 211 (called E211K) of the prion protein. Although the prevalence of this polymorphism is low, cattle carrying the K211 allele may be predisposed to rapid onset of BSE-H when exposed or to the potential development of a genetic BSE. This study was conducted to better understand the relationship between the K211 polymorphism and its effect on BSE phenotype. BSE-H from the US 2006 case was inoculated intracranially (IC) in one PRNP wild type (EE211) calf and one EK211 calf. In addition, one wild type calf and one EK211 calf were inoculated IC with brain homogenate from a US 2003 classical BSE case. All cattle developed clinical disease. The survival times of the E211K BSE-H inoculated EK211 calf (10 months) was shorter than the wild type calf (18 months). This genotype effect was not observed in classical BSE inoculated cattle (both 26 months). Significant changes in retinal function were observed in H-type BSE challenged cattle only. Cattle challenged with the same inoculum showed similar severity and neuroanatomical distribution of vacuolation and disease-associated prion protein deposition in the brain, though differences in neuropathology were observed between E211K BSE-H and classical BSE inoculated animals. Western blot results for brain tissue from challenged animals were consistent with the inoculum strains. This study demonstrates that the phenotype of E211K BSE-H remains stable when transmitted to cattle without the K211 polymorphism, and exhibits a number of features that differ from classical BSE in both wild type and heterozygous EK211 animals.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=326785
THURSDAY, JUNE 5, 2025
World Organisation for Animal Health (WOAH) downgrades UK’s BSE risk rating to negligible, what could go wrong?
https://bovineprp.blogspot.com/2025/06/world-organisation-for-animal-health.html
OIE Conclusions on transmissibility of atypical BSE among cattle
Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.
https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf
Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019
34 Scientific Commission/September 2019
3. Atypical BSE
The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.
4. Definitions of meat-and-bone meal (MBM) and greaves
http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf
The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion
https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article
Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/
Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/
Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate
Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata
Affiliations expand
PMID: 21266763
Abstract
A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.
https://pubmed.ncbi.nlm.nih.gov/21266763/
see full text;
https://www.niid.go.jp/niid/images/JJID/64/81.pdf
''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094
'Spontaneous mutation'
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.
https://www.nature.com/articles/srep11573
***> US Report, Scrapie, CWD, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026 <***
*** Grant Agreement number: 222887 ***
*** Project acronym: PRIORITY ***
*** Project title: Protecting the food chain from prions: shaping European priorities through basic and applied research Funding ***
Scheme: Large-scale integrating project Period covered: from Oct. 1, 2009 to Sept. 30, 2014
Name of the scientific representative of the project's co-ordinator1, Title and Organisation: Jesús R. Requena, Ph.D., Associate Professor, Department of medicine, University of Santiago de Compostela, Spàin. Tel: 34-881815464 Fax: 34-881815403 E-mail: jesus.requena@usc.es
Project website¡ Marcador no definido. address: www.prionpriority.eu
PRIORITY, PROJECT FINAL REPORT
*** 14) Concluding that atypical scrapie can transmit to Humans and that its strain properties change as it transmits between species ***
snip...
http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf
see;
https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html
https://nor-98.blogspot.com/
i’m hearing this imported beef is coming from Brazil and Argentina to the US under President Trump. this is a terrible, extremely risky idea, to try and shove Brazilian Beef down the throats of the US consumer, imho. Brazil has been caught trying to cover up mad cow disease for 2 years, cjd is out of control there, Argentina is questionable imo. this is a terrible idea on just human health risk factors alone.
i want to know where my beef comes from! i don’t want to risk eating Transmissible Spongiform Encephalopathy TSE Prion disease, neither should you, they can kill you, and Central and South America have no clue as to the BSE, Scrapie, CWD, Camel PrP TSE, Pig PrP TSE, especially Brazil…
with sad regards, terry
https://www.cattletoday.com/threads/bovine-spongiform-encephalopathy-bse-scrapie-cjd-tse-prion-in-south-america-2026-review.136659/
Bovine Spongiform Encephalopathy BSE, Scrapie, CWD, CJD, TSE prion Update September 2026
https://transmissiblespongiformencephalopathy.blogspot.com/2026/08/bovine-spongiform-encephalopathy-bse.html
https://prpsc.proboards.com/thread/238/scrapie-prion-update-september-2026
Singeltary comments and concerns…
Terry S. Singeltary Sr. Thank you for signing the Label Our Beef petition. Your signature has been recorded in support of restoring mandatory country-of-origin labeling (MCOOL) for beef. Together, we're sending a clear message to the White House and Congress: Consumers deserve to know where their beef comes from, and American cattle producers deserve the opportunity to distinguish their product in the marketplace.
Label Our Beef.
end…
Risk management strategies for bovine spongiform encephalopathy in South America
September 2010
International Journal of Risk Assessment and Management 14(3/4):254 DOI: 10.1504/IJRAM.2010.035270 Margaret A. WilsonShalu DarshanShalu DarshanDaniel KrewskiMichael G. TyshenkoMichael G. Tyshenko
Abstract and figures
Bovine spongiform encephalopathy (BSE) has never been reported in South America. As BSE began to be detected in other parts of the world, Argentina and Brazil were able to gain a larger share of the global demand for beef, with these two countries currently controlling one-third of the world export beef market. In both countries, the practice of pasturing cattle rather than feeding MBM has served to minimise the opportunity for BSE cycling, and early bans on the import of UK cattle coupled with minimal meat and bone meal (MBM) imports resulted in a very low external challenge. Specified risk material (SRM) regulations vary between countries in South America. Argentina enacted a ban on SRM in the feed chain in 2002; Brazil implemented a similar ban in early 2007. South American countries as a region had a lower risk of BSE entry, but higher cattle system instability than European Union countries.
Risk management strategies for BSE in South America
Conclusions
The implementation of preventive strategies for BSE in South America coupled with theadvantage of having a wealth of large natural grass plains used extensively for cattle grazing has played a major role in shielding the region from the influx of the disease. Argentina and especially Brazil enjoyed a boom in its beef export market as a result of BSE, with Brazil experiencing a 39% increase in beef exports in 2004 (USDA, 2005).The two countries benefited immensely from their favourable OIE and EFSA risk assessments at the time when BSE was being reported in many other countries. Other South American countries have begun to follow suit: Uruguay now exports as much beef as does Argentina, roughly 500 MT (US Department of Agriculture, 2008), and Paraguay shipped 183 MT in 2005 (Foreign Agriculture Service, USDA, 2006e).
Prior to the year 2007 the most evident difference between Argentina and Brazil in relation to BSE management was their respective policies for dealing with SRMs. Since January 2007 Brazil has implemented SRM controls. Argentina has excluded SRM from the feed chain since 2002. Brazil and Argentina recognised that there was considerable production of MBM in both respective countries intended mainly for domestic pig and poultry production; however, accidental cross-contamination could provide a means for amplification of BSE in cattle if the BSE agent were to gain entry into the national herd. This was true for Brazil prior to 2007, which, in addition to rendering SRM, imported more cattle from BSE-risk countries than did other South American countries prior to the import bans (Table 1). It must be emphasised that the predominance of pasturing in the Brazilian cattle industry and the rarity of co-farming act to minimise the risk of recycling of the infectious agent, even if it were present in imported cattle. Abetted by its willingness to provide beef raised without artificial hormones, Brazil has maintained a large share of the European beef market.
Both Argentina and Brazil were advised by EFSA to improve surveillance byincluding more at-risk animals in the testing program, such as fallen stock and emergency slaughters (European Food Safety Authority, 2005a, 2005b). The increasing orientation of the Brazilian beef industry towards the export market, prompted by a growing market share and shrinking domestic demand, makes the Brazilian industry vulnerable. The detection of a single case of BSE in the national herd could lead to catastrophic economici mpacts by limiting the beef trade in a reenactment of the situation that occurred in Canada after the detection of that country’s first BSE case in May 2003.
In the past, Argentina and Brazil have faced challenges to their beef export markets from foot and mouth disease (FMD) outbreaks. FMD outbreaks in the fall of 2005 in Brazil closed the borders of 58 markets, including Russia, Egypt, and the EU countries, to products from affected states (Meat and Livestock Australia, 2007b). Recently, the European Union banned Brazilian beef temporarily on grounds of unsatisfactory Brazilian cattle health checks for FMD (International Herald Tribune, 2008). Likewise in2001, Argentina dealt with an FMD crisis that severely challenged the beef export industry, resulting in intermittent border closures (World Organisation for Animal Health,2007b). The re-appearance of the disease after a protracted and intensive eradication program has led to a call for a ‘supranational’ surveillance system, possibly heralding broader harmonisation between countries of the region in the area of animal health (Can et al., 2004). Being mindful of the various means by which the beef export industry can be impacted negatively, regulatory bodies in these countries need to invest appropriate resources to control these emerging and reemerging cattle diseases.
(PDF) Risk management strategies for bovine spongiform encephalopathy in South America. Available from:
https://www.researchgate.net/publication/264815347_Risk_management_strategies_for_bovine_spongiform_encephalopathy_in_South_America
***> Brazil TSE Prion
Brazil 6 cases atypical BSE to date
Date: September 09, 2025 Report Number: BR2025-0024 Report Name: Livestock and Products Annual Country: Brazil Post: Brasilia Report Category: Livestock and Products Prepared By: Camila Aquino Approved By: Joseph Degreenia Report Highlights: Brazil is the second largest beef producing country and the largest beef exporting country in the world.
snip…
Animal Disease On September 9, 2024, MAPA published an Ordinance on norms of prevention and monitoring for bovine spongiform encephalopathy (BSE). Ordinance SDA/MAPA 1180/2024. To date, Brazil has only had six cases of BSE – all atypical. The country is considered as having insignificant risk for the disease per the World Organization for Animal Health (WOAH).
https://apps.fas.usda.gov/newgainapi/api/Report/DownloadReportByFileName?fileName=Livestock%20and%20Products%20Annual_Brasilia_Brazil_BR2025-0024
DIÁRIO OFICIAL DA UNIÃO Publicado em: 11/09/2024 | Edição: 176 | Seção: 1 | Página: 6 Órgão: Ministério da Agricultura e Pecuária/Secretaria de Defesa Agropecuária PORTARIA SDA/MAPA Nº 1.180, DE 9 DE SETEMBRO DE 2024 Estabelece as diretrizes do Programa Nacional de Encefalopatia Espongiforme Bovina para a aplicação de medidas oficiais de prevenção e vigilância.
https://www.gov.br/agricultura/pt-br/assuntos/insumos-agropecuarios/insumos-pecuarios/alimentacao-animal/portaria11802024.pdf
National Program for Surveillance and Prevention of Bovine Spongiform Encephalopathy - PNEEB
https://www.gov.br/agricultura/pt-br/assuntos/sustentabilidade/cesesp/eventos/vigilancia-das-sindromes-neurologicas-em-animais
Published on 06/21/2022 at 12:40 PM Updated on 06/21/2022 at 3:34 PM
https://www.gov.br/agricultura/pt-br/assuntos/sustentabilidade/cesesp/eventos/vigilancia-das-sindromes-neurologicas-em-animais/programa-nacional-de-vigilancia-e-prevencao-da-encefalopatia-espongiforme-bovina-pneeb.pdf/@@download/file
BRAZIL BSE START DATE 2023/01/18
BRAZIL BSE CONFIRMATION DATE 2023/02/22
BRAZIL BSE END DATE 2023/03/03
https://wahis.woah.org/#/in-review/4918
https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html
Brazil TSE Prion
FRIDAY, MARCH 3, 2023
Brazil MAPA OMSA Confirms BSE TSE Prion atypical H-type
https://animalhealthreportpriontse.blogspot.com/2023/03/brazil-mapa-omsa-confirms-bse-tse-prion.html
THURSDAY, FEBRUARY 23, 2023
Brazil Confirms BSE Mad Cow Case, Strain not known yet
https://animalhealthreportpriontse.blogspot.com/2023/02/brazil-confirms-bse-mad-cow-case-strain.html
TUESDAY, FEBRUARY 21, 2023
Brazil Suspected case of Bovine Spongiform Encephalopathy
https://animalhealthreportpriontse.blogspot.com/2023/02/brazil-suspected-case-of-bovine.html
''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094
FRIDAY, MARCH 17, 2023
***> Are Brazilian cervids at risk of prion diseases?
https://transmissiblespongiformencephalopathy.blogspot.com/2023/03/are-brazilian-cervids-at-risk-of-prion.html
THURSDAY, NOVEMBER 11, 2021
Brazil investigating two possible cases of mad cow disease in humans
https://creutzfeldt-jakob-disease.blogspot.com/2021/11/brazil-investigating-two-possible-cases.html
SATURDAY, NOVEMBER 13, 2021
Brazil Creutzfeldt Jakob Disease CJD TSE Prion Update 2021
https://creutzfeldt-jakob-disease.blogspot.com/2021/11/brazil-creutzfeldt-jakob-disease-cjd.html
TUESDAY, SEPTEMBER 07, 2021
Atypical Bovine Spongiform Encephalopathy BSE OIE, FDA 589.2001 FEED REGULATIONS, and Ingestion Therefrom
https://bse-atypical.blogspot.com/2021/09/atypical-bovine-spongiform.html
SATURDAY, SEPTEMBER 4, 2021
Brazil Confirms TWO More Cases of Mad Cow Disease BSE States of Mato Grosso and Minas Gerais
https://animalhealthreportpriontse.blogspot.com/2021/09/brazil-confirms-two-more-cases-of-mad.html
SATURDAY, JUNE 01, 2019
Brazil reports another cases of mad cow disease atypical BSE TSE Prion
https://bse-atypical.blogspot.com/2019/06/brazil-reports-another-cases-of-mad-cow.html
TUESDAY, MARCH 26, 2019
Joint Statement from President Donald J. Trump USA and President Jair Bolsonaro Brazil FOREIGN POLICY BSE TSE Prion aka mad cow disease
https://bseusa.blogspot.com/2019/03/joint-statement-from-president-donald-j.html
FRIDAY, NOVEMBER 03, 2017
First case of V180I rare mutation in a Brazilian patient with Creutzfeldt-Jakob disease
http://creutzfeldt-jakob-disease.blogspot.com/2017/11/first-case-of-v180i-rare-mutation-in.html
WEDNESDAY, DECEMBER 14, 2016
English lab confirms that mad cow disease in PR Brazil was atypical 4 years ago
https://bse-atypical.blogspot.com/2016/12/english-lab-confirms-that-mad-cow.html
TUESDAY, SEPTEMBER 27, 2016
Classical Scrapie Diagnosis in ARR/ARR Sheep in Brazil
Acta Scientiae Veterinariae, 2015. 43(Suppl 1): 69.
http://scrapie-usa.blogspot.com/2016/09/classical-scrapie-diagnosis-in-arrarr.html
MONDAY, AUGUST 1, 2016
USDA Announces Reopening of Brazilian Market to U.S. Beef Exports and the Potential for Transmissible Spongiform Encephalopathy TSE prion disease
http://madcowusda.blogspot.com/2016/08/usda-announces-reopening-of-brazilian.html
Cattle in Cacoal are slaughtered after eating prohibited food Cities
Friday, December 18, 2015 - 10:21
Chicken litter
Cattle in Cacoal are slaughtered after eating prohibited food
The Agrosilvopastoral Health Defense Agency of the State of Rondônia (Idaron) in Cacoal received a complaint from a teacher at the Agricultural Family School (EFA) that three cattle from a neighboring property accidentally entered the institution and ate "chicken litter," a prohibited food under federal law. The ban is one of the main preventive measures against bovine spongiform encephalopathy (BSE), also known as mad cow disease. The animals were slaughtered last week.
Idaron inspectors collected material ingested by cattle for laboratory testing So-called "chicken litter" is the result of grinding pine sawdust (used to line the floors of farm aviaries) and poultry feces.
Normative Instruction 41/2009 of the Ministry of Agriculture, Livestock, and Supply (MAPA) prohibits feeding ruminants products containing animal proteins and fats. The Normative Instruction also provides for fines for producers who feed their herds this type of feed.
Following the complaint, Idaron's technical team collected a sample of the material consumed by the cattle and confirmed, through examination in an official MAPA laboratory, that it was "chicken litter." The animals were identified and remained on their original rural property, and their movement was prohibited.
Because it was an accident, the owner of the animals, who also notified Idaron of the incident, was not fined; he was only notified to send the cattle for slaughter. Ney Carlos Dias de Azevedo, coordinator of the EEB State Prevention and Surveillance Program, explained that mad cow disease can be transmitted to humans through the ingestion of meat from infected animals. "There is no risk of contamination through contact with the animal," he added.
The coordinator also explained that since 2012, 404 cattle have been slaughtered in Rondônia as a safety measure for having consumed feed containing animal protein, and that in 2014, 132 inspections were carried out on farms that provide supplemental feed for cattle. "Idaron is on alert. The goal is to prevent the use of prohibited foods in ruminant feed, such as meat and bone meal and chicken litter."
The disease has already caused enormous losses to cattle ranching in Europe, in addition to causing deaths from ingesting contaminated meat. In Brazil, there have been only two recorded cases of mad cow disease, which are considered an atypical form of the disease (which can occur naturally in older animals). There is no record of ingestion of prohibited food.
http://www.rondoniagora.com/noticias/bovinos+de+cacoal+sao+abatidos+apos+ingerir+alimento+proibido+2015-12-18.htm
Friday, December 18, 2015 - 10:21
Poultry litter
Cacoal cattle are slaughtered after eating forbidden food
The Health Protection Agency Agrossilvopastoril the State of Rondônia (Idaron) Cacoal received a complaint from a professor at the Family Farm School (EFA) that three cattle of a neighboring property entered accidentally in the institution and were fed "poultry litter", food prohibited, according to federal law. The ban is one of the main measures to prevent bovine spongiform encephalopathy (BSE), known as mad cow disease. The animals were slaughtered last week.
snip...see full text;
https://translate.google.com/translate?hl=en&sl=auto&tl=en&u=http%3A%2F%2Fwww.rondoniagora.com%2Fnoticias%2Fbovinos%2Bde%2Bcacoal%2Bsao%2Babatidos%2Bapos%2Bingerir%2Balimento%2Bproibido%2B2015-12-18.htm
Friday, October 30, 2015
Brazil Agriculture minister to visit Saudi Arabia over BSE mad cow trade
http://bovineprp.blogspot.com/2015/10/brazil-agriculture-minister-to-visit.html
MONDAY, MAY 5, 2014
Brazil BSE Mad Cow disease confirmed OIE 02/05/2014
http://bovineprp.blogspot.com/2014/05/brazil-bse-mad-cow-disease-confirmed.html
Monday, May 5, 2014
Brazil 2nd BSE Mad Cow disease confirmed OIE 02/05/2014
http://bovineprp.blogspot.com/2014/05/brazil-bse-mad-cow-disease-confirmed.html
Thursday, April 24, 2014
Brazil investigates possible BSE mad cow case
http://bovineprp.blogspot.com/2014/04/brazil-investigates-possible-bse-mad.html
WEDNESDAY, JANUARY 29, 2014
Another Suspect case of Creutzfeldt-Jakob disease investigated in Brazil
http://creutzfeldt-jakob-disease.blogspot.com/2014/01/another-suspect-case-of-creutzfeldt.html
THURSDAY, SEPTEMBER 26, 2013
Brazil evaluate the implementation of health rules on animal by-products and derived products SRM BSE TSE PRION aka MAD COW DISEASE
http://bse-atypical.blogspot.com/2013/09/brazil-evaluate-implementation-of.html
Wednesday, December 19, 2012
Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE Risk (GBR) of Brazil
http://bse-atypical.blogspot.com/2012/12/scientific-report-of-european-food.html
***> Friday, December 07, 2012
***> ATYPICAL BSE BRAZIL 2010 FINALLY CONFIRMED OIE 2012
https://bse-atypical.blogspot.com/2012/12/atypical-bse-brazil-2010-finally.html
Updates on the WOAH activities in the field of TSEs
Natalie MOYEN Disease Status Officer
Status Department
May 14 th , 2024
Outline 1. Revised BSE standards (Terrestrial Code)
a) b) Transition process: where are we?
Official recognition of BSE risk status
i. Members/zones recognised as having a negligible or controlled BSE risk status
ii. New applications
iii. Annual reconfirmations
2. Revision of Scrapie standards (Terrestrial Code
Canada Trade Policy Monitoring Canada Bans Brazilian Beef Products 2001
https://apps.fas.usda.gov/newgainapi/api/Report/DownloadReportByFileName?fileName=Canada+Bans+Brazilian+Beef+Products_Ottawa_Canada_02-05-2001.pdf
Canada Livestock and Products Scientific Team to Assess BSE Status in Brazil 2001
https://apps.fas.usda.gov/newgainapi/api/Report/DownloadReportByFileName?fileName=Scientific+Team+to+Assess+BSE+Status+in+Brazil_Ottawa_Canada_02-14-2001.pdf
***> A critical perspective of Prion Disease surveillance in Brazil <***
2025 PRION 2025
Proceedings of the Prion 2025 - Advancing the understanding and treatment of prion diseases ISBN: 978-65-80968-48-0
A critical perspective of Prion Disease surveillance in Brazil
BARBOSA, BRENO JOSE ALENCAR PIRES 1MONTENEGRO, MARIA LUIZA VASCONCELOS 2CUNHA, JOSE ERITON GOMES DA 3 Vol 1, 2025 - 326431 Abstract Prion 2025
Abstract
Introduction: Prion Diseases (PrD) are a group of rapidly progressive dementias. Among its subtypes, Creutzfeldt-Jakob Disease (CJD) is the most common, affecting around 1 to 2 individuals per million inhabitants yearly. In 2005, Brazil's Ministry of Health (MH) initiated a surveillance program for CJD, creating a protocol to report the cases. Despite advances, the MH still struggles to make a reliable database to determine PrD profile in Brazil.
Objectives: To understand the Brazilian PrD surveillance system.
Methods: This is a retrospective and descriptive study based on the epidemiological records of CJD surveillance from 2005 to 2021 in the Ministry of Health's Epidemiological Bulletin published in 2022.
Results/Discussion: 1.576 suspected cases of CJD were reported, concentrated in the Southeast, South and Northeast regions of Brazil. Among the notifications, the following age groups predominated: 55 to 74 years (60.2%), 45 to 54 years (15%), and 75 to 85 years (11.8%). Suspected cases were mainly represented by women (53.6%), white individuals (61.4%), and residents of urban areas (90%). In this period, the expected number of cases for the Brazilian population would be 3.200.
Additionally to underreporting, the data shared by MH are limited due to the use of a database destined to mainly observe epidemic outbreaks and mismatches between official documents. Conclusion: Despite some progress since 2005, PrD surveillance in Brazil faces significant problems, due to the inaccurate treatment of these data and the lack of a specific database for CJD.
https://proceedings.science/prion-2025/papers/a-critical-perspective-of-prion-disease-surveillance-in-brazil?lang=en
SATURDAY, AUGUST 22, 2026
***> Brazil Creutzfeldt Jakob Disease CJD 547 confirmed cases in 16 years
https://creutzfeldt-jakob-disease.blogspot.com/2026/08/brazil-creutzfeldt-jakob-disease-cjd.html
https://prpsc.proboards.com/thread/233/brazil-cjd-confirmed-cases-years
***> Argentina TSE Prion
Argentina - Scrapie - Follow up report 1 GENERAL INFORMATION COUNTRY/TERRITORY OR ZONE
ZONE ANIMAL TYPE
TERRESTRIAL DISEASE CATEGORY
Listed disease EVENT ID
7429 DISEASE
Scrapie CAUSAL AGENT
Scrapie prion protein GENOTYPE / SEROTYPE / SUBTYPE
- START DATE
2025/02/20 REASON FOR NOTIFICATION
First occurrence in the country/territory DATE OF LAST OCCURRENCE
- CONFIRMATION DATE
2026/04/07 EVENT STATUS
On-going END DATE
- SELF-DECLARATION
NO REPORT INFORMATION REPORT NUMBER
Follow-up report 1 REPORT ID
FUR_183036 REPORT REFERENCE
- REPORT DATE
2026/05/12 REPORT STATUS
Validated NO EVOLUTION REPORT
https://wahis.woah.org/#/in-review/7429
WHO WAHIS WOAH Argentina - Scrapie - Immediate notification
CONFIRMATION DATE
2026/04/07 EVENT STATUS
https://wahis.woah.org/#/in-review/7429
First detection of classical scrapie in sheep imported from Paraguay in Santa Fe and Entre Ríos provinces, Argentina
MON 13 APR 2026
Source Ministry of Economy. Agriculture, Livestock, and Fisheries. National Food Safety and Quality Service (SENASA). Press release: For the first time, outbreaks of classical scrapie were detected in imported sheep. 11 Apr 2026 (in Spanish)
Key findings
On 11 Apr 2026, Argentina's National Service for Food Safety and Quality (SENASA) confirmed the country's first-ever detection of classical scrapie in three imported breeding sheep (Dorper and Santa Inés breeds) that died naturally without showing symptoms at establishments in the provinces of Santa Fe and Entre Ríos.
The affected sheep were imported from Paraguay in 2021 and 2022, underwent annual clinical inspections by SENASA veterinarians without any health issues, and were detected through active surveillance screening using ELISA testing, with confirmation by the Western Blot technique at a reference laboratory in Spain.
SENASA implemented official control and monitoring measures, including movement restrictions on affected production units, intensified surveillance, and sanitary containment protocols to prevent the spread of disease.
The detection was officially reported to the World Organisation for Animal Health (WOAH) and to Paraguay's National Service for Quality and Animal Health (SENACSA), and both countries agreed to deepen the epidemiological investigation.
Argentina is adapting its export certification to WOAH recommendations to maintain access to international markets, with safe products including embryos, hides, skins, gelatin, collagen, tallow, wool, and sheep meat with risk tissues removed. It is also proposing specific mitigation measures for live animals, protein meals, and dairy products.
Epidemiological analysis and public health impact
This detection marks a fundamental shift in Argentina's animal health status, moving the country from scrapie-free to disease-present and requiring the establishment of new surveillance infrastructure and baseline monitoring systems. Identification through active surveillance screening, rather than clinical presentation, demonstrates the effectiveness of Argentina's post-import monitoring protocols, yet also reveals that subclinical infections can persist undetected for years despite annual veterinary inspections.
The vertical transmission pattern at birth poses a significant risk of within-flock amplification, particularly because the affected animals were breeding stock imported 4–5 years ago, which could have allowed multiple lambing cycles before detection. The absence of clinical signs until natural death suggests that other subclinical carriers may exist in the affected flocks and possibly in offspring, necessitating comprehensive herd testing and assessment of genetic susceptibility.
The geographic distribution across two provinces (Santa Fe and Entre Ríos) and the common Paraguayan origin of the imports indicate a point-source introduction with the potential for wider dissemination through breeding-stock sales or animal movements before detection. The restriction measures now in place will be critical to preventing establishment in Argentina's broader sheep population, which could have substantial economic implications for the country's wool and meat export industries.
snip…
https://beaconbio.org/en/report/?search=&locations=e768e738-503e-4788-b427-dac003976695&reportid=327edbf5-7ae1-4d50-9316-43660615345c&filtersOpen=true
Outbreaks of classical scrapie were detected in imported sheep for the first time.
SENASA reported the finding in breeding specimens belonging to establishments in the provinces of Santa Fe and Entre Ríos.
April 11, 2026
The National Service for Agrifood Health and Quality (SENASA) confirmed the positive result for classical scrapie in imported breeding sheep , after the natural death without associated symptoms of three animals was recorded in establishments in Santa Fe and Entre Ríos.
The sheep involved had been imported in 2021 and 2022 from Paraguay, in accordance with the sanitary requirements and the current International Veterinary Certificate model, passing the required post-entry controls.
https://www.argentina.gob.ar/noticias/por-primera-vez-se-detectaron-brotes-de-scrapie-clasico-en-ovinos-importados
FINAL REPORT OF AN AUDIT CARRIED OUT IN ARGENTINA FROM 21 NOVEMBER 2023 TO 1 DECEMBER 2023 IN ORDER TO EVALUATE THE IMPLEMENTATION OF OFFICIAL CONTROLS ON ANIMAL BY- PRODUCTS (ABP) AND DERIVED PRODUCTS (DP)
Executive Summary
This report describes the outcome of an audit in Argentina carried out from 21 November to 1 December 2023. The audit was part of the European Commission’s Directorate-General for Health and Food Safety’s published work programme.
The objective of the audit was to assess whether the official control system over the production of rendered fat (tallow) not intended for human consumption and destined for export to the European Union (EU), as well as related certification arrangements, are sufficient to allow for reliable attestation by the responsible competent authority that the EU requirements specified in the model EU health certificate in Chapter 10(B) of Annex XV to Commission Regulation (EU) No 142/2011 are complied with. This was the first time that the Commission services have carried out an audit on the subject of animal by-products (ABP) and derived products in Argentina. The report concludes that the official control system governing the production and certification of ABP for the EU market is not fit for its intended purpose. It results in the Argentinian competent authority not being in a position to reliably verify and certify compliance of tallow with the vast majority of requirements relevant for its production and export to the EU including identifying the category of the ABP used for its manufacture, the processing method used and the marking of the product with glycerol triheptanoate.
Due to the severity and scale of the non-compliances identified during the audit, the competent authority indicated its intention to revoke the EU export authorisations of all of the tallow processing plants and delist them from TRACES-NT, pending the implementation of corrective actions.
The report contains two recommendations to the Argentinian competent authority to address the identified gaps.
snip…
Conclusions on official certification
Given the gaps in the official controls performed and the absence of certifying officers’ direct knowledge of, or evidence for the category of the raw material used for the production of tallow, the processing method used, and the use of GTH and its content, the competent authority’s attestations of compliance with most of requirements referred to in the EU health certificate are unreliable.
6 OVERALL CONCLUSION
The official control system governing the production and certification of ABP for the EU market is not fit for its intended purpose. It results in the Argentinian competent authority not being in a position to reliably verify and certify compliance of tallow with the vast majority of requirements relevant for its production and export to the EU including identifying the category of the ABP used for its manufacture, the processing method used and the marking of the product with glycerol triheptanoate.
https://ec.europa.eu/food/audits-analysis/audit-report/download/16445
1994 Sep;13(3):801-36. doi: 10.20506/rst.13.3.807.
Bovine spongiform encephalopathy surveillance in Argentina
[Article in English, Spanish] A A Schudel 1 , B J Carrillo, E J Gimeno, E L Weber, J Blanco Viera, C van Gelderen, E Ulloa, A Nader, B G Cané Affiliations expand PMID: 7949355 DOI: 10.20506/rst.13.3.807
Abstract
Bovine spongiform encephalopathy (BSE) is a new disease of cattle first described in the United Kingdom in November 1986. BSE belongs to the scrapie-related group of diseases. The epidemiological studies performed in the United Kingdom demonstrate that the BSE epidemic was caused by feeding cattle with ruminant-derived protein contaminated by a scrapie-like agent. Until June 1994, the disease had been detected in indigenous cattle in Ireland, Switzerland and France. Three cases reported in Germany, two in the Sultanate of Oman, and single cases in the Falkland Islands (Islas Malvinas), Denmark, Portugal and Canada occurred in animals imported from the United Kingdom. Several countries have implemented surveillance programmes analysing the risk factors involved in the epidemic. An analysis of risk factors conducted in Argentina shows that it is highly unlikely that BSE or scrapie exist in the country, or will arise via feed in the future. As a continuation of the analysis of risk factors, a surveillance programme was implemented in the field and in abattoirs. Specialised personnel were trained in the clinical, histopathological and biochemical detection of the disease through a network of laboratories which covered 85% of the total cattle population and 100% of the high-risk group (dairy cows over five years of age). By using a statistical procedure with reference to the bovine population in nine provinces, 1,019 brains from animals belonging to the high-risk group were selected and studied by histopathological and biochemical analyses for BSE detection. The results were negative in all cases. It can be concluded from this analysis (with a sensitivity of detection of 2.95 per 1,000, and 95% statistical confidence) that Argentina may be regarded as BSE-free, and that the importation of infected animals or by-products may represent the sole potential source of introduction of BSE infection into the country in the future.
https://pubmed.ncbi.nlm.nih.gov/7949355/
[Analysis of risk factors associated with bovine spongiform encephalopathy in Argentina]
Cané BG, Gimeno EJ, Manetti JC, Van Gelderen C, Ulloa E, Schudel AA. Rev Sci Tech. 1993 Dec;12(4):1203-34. PMID: 8312622Spanish.
https://pubmed.ncbi.nlm.nih.gov/8312622/
National MCOOL Coalition Launches Petition Calling on White House, Congress to Label Our Beef
WASHINGTON, Sept. 1, 2026
i want to know where my beef comes from! i don’t want to risk eating Transmissible Spongiform Encephalopathy TSE Prion disease, neither should you, they can kill you, and Central and South America have no clue as to the BSE, Scrapie, CWD, Camel PrP TSE, Pig PrP TSE!
https://www.cattletoday.com/threads/bovine-spongiform-encephalopathy-bse-scrapie-cjd-tse-prion-in-south-america-2026-review.136659/
***> atypical BSE and atypical Scrapie <***
Atypical BSE in cattle
THE recent diagnosis of two atypical bovine spongiform encephalopathy (BSE) cases in Great Britain (March 2023 in Cornwall and December 2024 in Dumfries and Galloway) and one in the Republic of Ireland (in November 2023) warrants a reminder about this notifiable disease.
Since 2005, a total of 17 cases have been detected in Great Britain.1 Unlike classical BSE, which resulted in over 180,000 cases in Great Britain and was predominantly associated with the consumption of feed contaminated with the BSE agent, and where the last case was confirmed in Ayrshire in May 2024, atypical BSE is believed to be a spontaneous disease in cattle found in approximately one in 1,000,000 tested cattle based on French data,2 similar to the sporadic Creutzfeldt- Jakob disease in people. There is currently no evidence that atypical BSE causes a disease in people, although it can be transmitted experimentally to other species by intracerebral inoculation, including primates.3–5 The World Organisation for Animal Health does not include atypical BSE in its geographical BSE risk status assessment.
Despite differences in terms of epidemiological, molecular and biological phenotype compared with classical BSE, atypical BSE is currently treated as if it were classical BSE in accordance with EU and UK legislation: once a case is identified, all cohort animals born and reared with the affected animal during the first 12 months of its life, and all offspring born within 24 months of its clinical onset, are culled and tested for BSE, which does seem to be at odds with the hypothesis that it is a spontaneous disease. This is more a precautionary measure to maintain confidence in the beef trade and protect consumers while more knowledge about this disease is obtained.
Almost all current knowledge on atypical BSE is based on experimental infection because this spontaneous
VET RECORD | 29 March–12 April 2025
disease has generally only been found in aged downer cows, which is difficult to replicate experimentally in the host species. Intracerebral inoculation of brain tissue from an affected cow causes disease in cattle in less than two years, unlike the natural disease that usually occurs in animals over eight years of age.
The vast majority of cases have been identified by active monitoring of fallen stock or emergency slaughter of cattle, where only the brain sample of various stages of autolysis is generally available. Little is known of where the atypical BSE agent can be found in natural disease, other than in the brain, because all the cases confirmed have been identified after death through active surveillance, by which time most peripheral tissue has been disposed of. Limited material from a single case of a naturally affected cow was tested in Italy by mouse bioassay, which found infectivity in muscle.6 In experimental disease generated by intracerebral inoculation of cattle, infectivity can be detected in the brain and spinal cord, ganglia, peripheral nerves and skeletal muscles, similar to classical BSE, but not in peripheral lymphoid tissue.6–8
Early reporting of clinical suspects is needed so that the live animal or the whole carcase can be delivered to an APHA regional laboratory for tissue sampling. This is made more difficult due to the subtlety of clinical signs based on experimental disease. Clinical cases may not be as over- reactive or nervous as classical BSE cases; some may, in fact, be dull, but what most cases have in common is that they have difficulty getting up and eventually end up as downer cows, and only the clinical history may reveal some prior behavioural or locomotor changes. High creatinine kinase serum levels and nibbling in response to scratching the tail head or back were some features in experimental disease,8, 9 but it is not known whether this is also seen in natural disease.
In general, BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment, where the blood results do not support the presence of a metabolic disease and where the cause cannot be determined with confidence.
Since BSE is a notifiable disease, suspected cases of BSE in Great Britain must be reported to the local APHA office.
Changes are imminent in the reporting of fallen stock cattle, which will require the owner to state whether the animal displayed signs of changes in behaviour, sensation or locomotion before death, in addition to the likely cause of death or disease. This is to obtain a better profile of the clinical history, if cattle are retrospectively diagnosed as BSE cases, which has happened in all BSE cases confirmed since 2010: none has been reported as a clinical suspect.
“BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment”
Timm Konold, TSE lead scientist
Brenda Rajanayagam, workgroup leader for the data systems group
APHA Weybridge, New Haw, Addlestone, Surrey KT15 3NB email: timm.konold@apha.gov.uk
Keith Meldrum, former chief veterinary officer The Orchard, Swaynes Lane, Guildford, Surrey GU1 2XX
References
1 APHA. Cattle: TSE surveillance statistics. Overview of Great Britain statistics. 2025. https://bit.ly/4ho5Nds (accessed 19 March 2025)
Atypical BSE In Cattle
https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1002/vetr.5400?campaign=woletoc
see latest on BSE TSE Prion EU;
https://bse-atypical.blogspot.com/2026/04/ireland-central-veterinary-research.html
Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle
Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *
Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.
*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca
Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.
Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.
Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.
Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.
Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.
Presentation Type: Oral Presentation
Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute
Grant Number: ALMA/APRI: 201400006, HC 414250
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE)
Component 6: Transmissible Spongiform Encephalopathies (TSEs)
Problem Statement 6A: Determine pathobiology of prion strains.
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE).
Virus and Prion Research Unit, National Animal Disease Center, Ames, Iowa
Classical BSE (C-BSE) is a prion disease of cattle that was responsible for the "mad cow disease" epizootic in Europe in the 1980s. C-BSE was determined to cause the human prion disease vCJD. Since then, atypical spontaneous strains of BSE were identified. H-BSE is one of those strains. Much research has explored the origins of C-BSE, and strain emergence from atypical H-BSE is one hypothesis. An H-BSE case was determined to have a germline mutation, an E211K substitution in the prion protein gene, which is analogous to a hereditary human prion disease. ARS scientists in Ames, Iowa reported the transmission of H-BSE from cattle, with and without the germline prion protein amino acid substitution, to cattle with various prion genotypes: EE211 (wild-type), EK211, and KK211. Results indicated a significantly shorter incubation period in K containing cattle compared to prion wild-type cattle. The scientists also explored the possibility that the C-BSE strain might have occurred after serial passages of EK211 and KK211 containing H-BSE in cattle, but results did not support this concept. This information is important to prion researchers, veterinary diagnostic laboratories, and those involved with establishing regulatory guidelines.
https://www.ars.usda.gov/ARSUserFiles/np103/AnnualReports/Final%20NP103%20FY2024%20Annual%20Report.updated%205.30.25.pdf
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research
Title: Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation
Author item Greenlee, Justin item Cassmann, Eric item MOORE, SARA JO - Oak Ridge Institute For Science And Education (ORISE) item WEST GREENLEE, HEATHER - Iowa State University
Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 6/24/2022 Publication Date: 9/16/2022 Citation: Greenlee, J.J., Cassmann, E.D., Moore, S., West Greenlee, H.M. 2022.
Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation.
Prion 2022 Conference abstracts: pushing the boundaries. 16(1):150. https://doi.org/10.1080/19336896.2022.2091286.
DOI: https://doi.org/10.1080/19336896.2022.2091286
Interpretive Summary:
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure. Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n=3) or from the US 2006 case with the E211K polymorphism (n=4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation. Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57-4.0) in the brainstem, and IHC demonstrated PrPSc throughout the brain. All cattle in the EE211 recipient group remain asymptomatic for the duration of the experiment (approximately 7 years post-inoculation).
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351
Highlights
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351
Title: A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation
Author item MOORE, S - Orise Fellow item WEST GREENLEE, M - Iowa State University item Smith, Jodi item Vrentas, Catherine item Nicholson, Eric item Greenlee, Justin
Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 8/30/2016 Publication Date: 9/15/2016 Citation: Moore, S.J., West Greenlee, M.H., Smith, J.D., Vrentas, C.E., Nicholson, E.M., Greenlee, J.J. 2016.
A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation.
Frontiers in Veterinary Science. 3:78. Interpretive
Summary: Cases of bovine spongiform encephalopathy (BSE) or mad cow disease can be subclassified into at least 3 distinct disease forms with the predominate form known as classical BSE and the others collectively referred to as atypical BSE. Atypical BSE can be further subdivided into H-type and L-type cases that are distinct from classical BSE and from each other. Both of the atypical BSE subtypes are believed to occur spontaneously, whereas classical BSE is spread through feeding contaminated meat and bone meal to cattle. Work by other research groups suggests that the stability of the distinguishing features of atypical BSE cases (phenotypical stability) can change to closely resemble classical BSE after experimental passage implicating atypical BSE as a possible origin of classical BSE. Interestingly, one case of H-type BSE in the US was associated with an inherited mutation in the prion protein gene referred to as E211K. The purpose of this work was to compare wild type and cattle with the E211K mutation after experimental inoculation with either classical BSE or H-BSE from the original E211K case. This study demonstrates that the disease features of E211K BSE-H remain stable when transmitted to cattle without the K211 polymorphism. In addition, passage of classical BSE to cattle with the K211 polymorphism results in disease with features consistent with classical BSE and not a switch to atypical BSE-H as a result of the K211 polymorphism. As the origin of classical, feedborne BSE remains unknown and low numbers of atypical BSE are diagnosed each year, parties with interest in the cattle and beef industries and regulatory officials responsible for safe feeding practices of cattle will be interested in this work.
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (BSE-H) was diagnosed in a cow that was associated with a heritable polymorphism in the bovine prion protein gene (PRNP) resulting in a lysine for glutamine amino acid substitution at codon 211 (called E211K) of the prion protein. Although the prevalence of this polymorphism is low, cattle carrying the K211 allele may be predisposed to rapid onset of BSE-H when exposed or to the potential development of a genetic BSE. This study was conducted to better understand the relationship between the K211 polymorphism and its effect on BSE phenotype. BSE-H from the US 2006 case was inoculated intracranially (IC) in one PRNP wild type (EE211) calf and one EK211 calf. In addition, one wild type calf and one EK211 calf were inoculated IC with brain homogenate from a US 2003 classical BSE case. All cattle developed clinical disease. The survival times of the E211K BSE-H inoculated EK211 calf (10 months) was shorter than the wild type calf (18 months). This genotype effect was not observed in classical BSE inoculated cattle (both 26 months). Significant changes in retinal function were observed in H-type BSE challenged cattle only. Cattle challenged with the same inoculum showed similar severity and neuroanatomical distribution of vacuolation and disease-associated prion protein deposition in the brain, though differences in neuropathology were observed between E211K BSE-H and classical BSE inoculated animals. Western blot results for brain tissue from challenged animals were consistent with the inoculum strains. This study demonstrates that the phenotype of E211K BSE-H remains stable when transmitted to cattle without the K211 polymorphism, and exhibits a number of features that differ from classical BSE in both wild type and heterozygous EK211 animals.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=326785
THURSDAY, JUNE 5, 2025
World Organisation for Animal Health (WOAH) downgrades UK’s BSE risk rating to negligible, what could go wrong?
https://bovineprp.blogspot.com/2025/06/world-organisation-for-animal-health.html
OIE Conclusions on transmissibility of atypical BSE among cattle
Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.
https://www.oie.int/fileadmin/SST/adhocreports/Bovine%20spongiform%20encephalopathy/AN/A_AhG_BSEsurv_RiskAss_Mar2019.pdf
Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019
34 Scientific Commission/September 2019
3. Atypical BSE
The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.
4. Definitions of meat-and-bone meal (MBM) and greaves
http://web.oie.int/downld/PROC2020/A_SCAD_Sept2019.pdf
The L-type BSE prion is much more virulent in primates and in humanized mice than is the classical BSE prion, which suggests the possibility of zoonotic risk associated with the L-type BSE prion
https://wwwnc.cdc.gov/eid/article/16/7/09-1882_article
Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5324790/
Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3310119/
Atypical L-type bovine spongiform encephalopathy (L-BSE) transmission to cynomolgus macaques, a non-human primate
Fumiko Ono 1, Naomi Tase, Asuka Kurosawa, Akio Hiyaoka, Atsushi Ohyama, Yukio Tezuka, Naomi Wada, Yuko Sato, Minoru Tobiume, Ken'ichi Hagiwara, Yoshio Yamakawa, Keiji Terao, Tetsutaro Sata
Affiliations expand
PMID: 21266763
Abstract
A low molecular weight type of atypical bovine spongiform encephalopathy (L-BSE) was transmitted to two cynomolgus macaques by intracerebral inoculation of a brain homogenate of cattle with atypical BSE detected in Japan. They developed neurological signs and symptoms at 19 or 20 months post-inoculation and were euthanized 6 months after the onset of total paralysis. Both the incubation period and duration of the disease were shorter than those for experimental transmission of classical BSE (C-BSE) into macaques. Although the clinical manifestations, such as tremor, myoclonic jerking, and paralysis, were similar to those induced upon C-BSE transmission, no premonitory symptoms, such as hyperekplexia and depression, were evident. Most of the abnormal prion protein (PrP(Sc)) was confined to the tissues of the central nervous system, as determined by immunohistochemistry and Western blotting. The PrP(Sc) glycoform that accumulated in the monkey brain showed a similar profile to that of L-BSE and consistent with that in the cattle brain used as the inoculant. PrP(Sc) staining in the cerebral cortex showed a diffuse synaptic pattern by immunohistochemistry, whereas it accumulated as fine and coarse granules and/or small plaques in the cerebellar cortex and brain stem. Severe spongiosis spread widely in the cerebral cortex, whereas florid plaques, a hallmark of variant Creutzfeldt-Jakob disease in humans, were observed in macaques inoculated with C-BSE but not in those inoculated with L-BSE.
https://pubmed.ncbi.nlm.nih.gov/21266763/
see full text;
https://www.niid.go.jp/niid/images/JJID/64/81.pdf
''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=353094
'Spontaneous mutation'
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.
https://www.nature.com/articles/srep11573
***> US Report, Scrapie, CWD, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026 <***
*** Grant Agreement number: 222887 ***
*** Project acronym: PRIORITY ***
*** Project title: Protecting the food chain from prions: shaping European priorities through basic and applied research Funding ***
Scheme: Large-scale integrating project Period covered: from Oct. 1, 2009 to Sept. 30, 2014
Name of the scientific representative of the project's co-ordinator1, Title and Organisation: Jesús R. Requena, Ph.D., Associate Professor, Department of medicine, University of Santiago de Compostela, Spàin. Tel: 34-881815464 Fax: 34-881815403 E-mail: jesus.requena@usc.es
Project website¡ Marcador no definido. address: www.prionpriority.eu
PRIORITY, PROJECT FINAL REPORT
*** 14) Concluding that atypical scrapie can transmit to Humans and that its strain properties change as it transmits between species ***
snip...
http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf
see;
https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html
https://nor-98.blogspot.com/
Block D: Prion epidemiology
Studies on atypical scrapie were identified as a key element of this block, given the potential risk associated to this agent. We studied the permeability of Human, bovine and porcine species barriers to atypical scrapie agent transmission. Experiments in transgenic mice expressing bovine, porcine or human PrPC suggest that this TSE agent has the intrinsic ability to propagate across these species barriers including the Human one. Upon species barrier passage the biological properties and phenotype of atypical scrapie seem to be altered. Further experiments are currently ongoing (in the framework of this project but also in other projects) in order to: (i) characterize the properties of the prion that emerged from the propagation of atypical scrapie in tg Hu; (ii) to confirm that the phenomena we observed are also true for atypical scrapie isolates other than the ones we have studied.
In parallel, studies in sheep have concluded that:
*** Atypical scrapie can be transmitted by both oral and intracerebral route in sheep with various PRP genotypes
*** Low but consistent amount of infectivity accumulates in peripheral tissue (mammary gland, lymph nodes, placenta, skeletal muscles, nerves) of sheep incubating atypical scrapie.
*** The combination of data from all our studies leads us to conclude that:
*** Atypical scrapie passage through species barriers can lead to the emergence of various prions including classical BSE (following propagation in porcine PRP transgenic mice).
*** Atypical scrapie can propagate, with a low efficacy, in human PrP expressing mice. This suggests the existence of a zoonotic potential for this TSE agent.
snip...
We advance our main conclusions and recommendations, in particular as they might affect public policy, including a detailed elaboration of the evidence that led to them. Our main recommendations are:
a. The issue of re-introducing ruminant protein into the food-chain The opinion of the members of Priority is that sustaining an absolute feed ban for ruminant protein to ruminants is the essential requirement, especially since the impact of non-classical forms of scrapie in sheep and goats is not fully understood and cannot be fully estimated. Therefore, the consortium strongly recommends prohibiting re-introduction of processed ruminant protein into the food-chain. Arguments in support of this opinion are:
• the large (and still uncharacterized) diversity of prion agents that circulate in animal populations;
• the uncertainties related to prion epidemiology in animal populations;
• the unknown efficacy of industrial processes applied to reduce microbiological risk during processed animal protein (PAP) production on most prion agents; • the intrinsic capacity of prions to cross interspecies transmission barriers; • the lack of sensitive methodology for identifying cross contamination in food.
• the evolution of natural food chains in nature (i.e. who eats whom or what) has generated an efficient barrier preventing, to some extent, novel prion epidemies and that this naturally evolved ecology should be respected.
The consortium is also hesitant to introduce processed ruminant proteins into fish food considering the paucity of data on prion infections in fishes and sea animals including those of mammalian origin, and the risk of establishing an environmental contamination of the oceans that cannot be controlled.
b. Atypical prion agents and surveillance
Atypical prion agents (see below) will probably continue to represent the dominant form of prion diseases in the near future, particularly in Europe.
*** Atypical L-type BSE has clear zoonotic potential, as demonstrated in experimental models.
*** Similarly, there are now some data that seem to indicate that the atypical scrapie agent can cross various species barriers.
*** Moreover, the current EU policy for eradicating scrapie (genetic selection in affected flocks) is ineffective for preventing atypical scrapie.
*** The recent identification of cell-to-cell propagation and the protein-encoded strain properties of human neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, suggest that they bear the potential to be transmissible even if not with the same efficiency as CJD. More epidemiological data from large cohorts are necessary to reach any conclusion on the impact of their transmissibility on public health. Re-evaluations of safety precautions may become necessary depending on the outcome of these studies. In that context it would appear valuable
• to develop knowledge related to the pathogenesis and inter-individual transmission of atypical prion agents in ruminants (both intra- and inter-species)
• to improve the sensitivity of detection assays that are applied in the field towards this type of agent
• to maintain a robust surveillance of both animal and human populations
c. The need for extended research on prions
Intensified searching for a molecular determinants of the species barrier is recommended, since this barrier is a key for many important policy areas - risk assessment, proportional policies, the need for screening of human products and food. In this respect, prion strain structural language also remains an important issue for public health for the foreseeable future. Understanding the structural basis for strains and the basis for adaptation of a strain to a new host will require continued fundamental research. Prions maintain a complex two-way relationship with the host cell and fundamental research is needed on mechanisms for their transmission, replication and cause of nervous system dysfunction and death.
Early detection of prion infection, ideally at preclinical stage, also remains crucial for development of effective treatment strategies in humans affected by the disease.
Position of the Priority consortium
Nearly 30 years ago, the appearance in the UK of Bovine Spongiform Encephalopathy (BSE) quickly brought the previously obscure “prion diseases” to the spotlight. The ensuing health and food crises that spread throughout Europe had devastating consequences. In the UK alone, there were more than 36,000 farms directly affected by BSE and the transmission of BSE prions to humans via the food chain has caused over 200 people in Europe to die from variant Creutzfeldt-Jakob disease (vCJD) (http://www.cjd.ed.ac.uk
Origins of prion epidemies
Classical BSE now appears to be under control, with 18 EU Member States having achieved the World Organisation for Animal Health (Office International Epizooties) „negligible risk‟ status (May 2014; http://www.oie.int/en/animal-health-in-the-world/official-disease-status/bse/list-of-bse-risk-status/), and the remaining MS assessed as „controlled‟ risk. Of note, research, including EU-funded research, has played a key role in this success: while the origin of the infection was never defined, the principle driver of the epidemic was identified as prions in Meat and Bone Meal (MBM). Tests based on prion protein-specific antibodies were developed, allowing detection of infected animals, and a better understanding of disease pathogenesis and the distribution of infectivity in edible tissues; experimental investigation of transmission barriers between different species allowed a rational estimation of risks, etc. All of this led to the implementation of rational and effective policies, such as the MBM ban to protect the animal feed chain, and the Specified Risk Material (SRM) regulations to protect the human food chain.
In spite of this progress, prions are still a threat. Epidemiological re-assessment indicates that the ∼10 year incubation period separating the peaks of the BSE and the vCJD epidemics is probably too short. In addition, results from a large number of human tonsil and appendix analyses in the UK suggest that there may be a high number of asymptomatic individuals who are positive for the disease-associated conformer prion protein PrPSc. While vCJD is the only form of human prion disease that has been consistently demonstrated to have lymphoreticular involvement, there has been no systematic investigation of lymphoid tissue in cases with other prion diseases.
The human prion problem
The clinical cases of vCJD identified to date have all shared a common PrP genotype (M129M), although one pre-clinical case was confirmed as an M129V heterozygote, and it has been mooted that perhaps only the M129M proportion of the population is susceptible. However, in the UK appendix study, PrP accumulation was described in samples representing every codon 129 genotype, raising the possibility that genotype does not confer resistance but instead modulates incubation period. Apart from the two UK studies, the lymphoid tissues of non-CJD patients have not been examined for the presence of PrPSc, so, these cases may not solely represent pre-clinical vCJD, but also other forms of prion disease.
Recent experiments in highly susceptible mouse models indicate the presence of infectivity in blood or blood components at late disease stages in sporadic CJD. The significance of this experimental finding for humans has to be explored in more detail and, at the present time, there is no evidence for the transmission of prions via blood in sporadic CJD. However a likely scenario is that all those with signs of infection or abnormal PrP accumulation in peripheral tissue could have infective blood, posing the risk for transmission via blood products, which has been clearly demonstrated in experimental models, and confirmed in several cases of vCJD in man. Altogether, these data clearly demonstrate the potential risk of a second wave of vCJD, particularly when the number people identified with lymphoid accumulation of PrPSc (16/32,411) gives a prevalence estimate in the UK of 493 per million, much higher than the number of clinical cases seen to date.
The animal prion problem
An increasing number of reports on cases of “atypical” BSE in cattle throughout the EU and beyond may lead to a new epidemic, particularly since we still do not understand all factors determining the species barrier. Ovine scrapie is another concern, because it could mask ovine BSE, presumably transmissible to humans. Scrapie is endemic and not likely to be eradicated soon, although current control measures are effective at greatly reducing disease incidence. Atypical forms, which may be spontaneous, are not affected by these control measures and these forms of disease will persist in the global animal population. The low prevalence of these disease forms makes effective surveillance very challenging. However, there is a clear risk attendant on ignoring these cases without an understanding of their possible zoonotic potential, particularly when most forms of human disease have no established aetiology. In summary, atypical cases of BSE and scrapie presently clearly outnumber classical cases in cattle and sheep in all member states.
We will highlight the state-of-the-art knowledge and point out scientific challenges and the major questions for research. Strategic objectives and priorities in Europe in the future for research that aims to control, eliminate or eradicate the threat posed by prions to our food and health are also indicated.
The Priority project has focused on 4 themes, namely the structure, function, conversion and toxicity of prions; detection of prions; mechanisms of prion transmission and spreading and epidemiology of prion diseases. This paper summarizes the opinions/positions reached within these themes at the end of the project.
http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf
see;
https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html
Transmission of scrapie prions to primate after an extended silent incubation period
*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.
*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160
O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations
*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases.
==============
PRION 2015 CONFERENCE
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/
PRION 2016 TOKYO
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-68961933-690X
WS-01: Prion diseases in animals and zoonotic potential
***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.
***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.
***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20
Title: Transmission of scrapie prions to primate after an extended silent incubation period)
*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.
*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160
Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014)
Ru G1 ., Pocchiari M2 ., Bertolini S. 1, Pite L.1 , Puopolo M.2 , Ladogana A.2 , Perrotta M.G.3 , Meloni D 1 . (1) National reference center for the study and research on animal encephalopathies and comparative neuropathologies (CEA). Experimental Zooprophylactic Institute of Piemonte, Liguria and Valle d'Aosta, Torino, Italy.
(2) Department of Cellular Biology and Neuroscience, Istituto Superiore di Sanità, Roma, Italy. (3) Office 3 National center for the fight and emergency against animal diseases. Ministry of Health, Roma, Italy.
Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure.
Materials and Methods: National data from prion disease surveillance in humans (sporadic CJD) and small ruminants (CS) in Italy were utilized. A descriptive geographic analysis was conducted for each disease individually. Subsequently, an ecological study was performed to compare the occurrence of both diseases at the district and regional levels. Standardized incidence ratios (SIR), adjusted for confounders, were calculated for CJD and CS by district and region, respectively, representing the outcome and proxy of exposure. Considering a possible long incubation period of CJD, two study periods were analysed: 2010-2014 for CJD and 2002-2006 for CS. Eight alternative linear regression models were developed using SIR in humans as the dependent variable and SIR in sheep as the independent variable. These models varied in the scale of SIR data (continuous vs. categorical), geographical level (district vs. region), and the potential past exposure of sheep in specific areas to a known source of infection (via a contaminated vaccine).
Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased.
Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.
Funded by: Italian Ministry of Health Grant number: Realizzazione del programma epidemiologico finalizzato a dare evidenza del potenziale zoonotico delle TSE animali diverse dalla BSE. Prot. N. 0018730-17/07/2015-DGSAFCOD_UO-P
''Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.''
Meeting-book-final-version prion 2023 Prion 2023 Congress Organizing Committee and the NeuroPrion Association, we invite you to join us for the International Conference Prion2023 from 16-20 October 2023 in Faro, Portugal.
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https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene
Waqas Tahir , Sandor Dudas , Renee Anderson , Jianmin Yang , Sarah Bogart , Kristina Santiago-Mateo, Yuanmu Fang & Roberta Quaghebeur
Pages 36-49 | Received 20 Feb 2025, Accepted 22 May 2025, Published online: 04 Aug 2025 Cite this article https://doi.org/10.1080/19336896.2025.2511933
ABSTRACT
Bovine Spongiform Encephalopathy (BSE) is a fatal neurodegenerative disease in cattle which can be either classical BSE (C-BSE) or atypical BSE (including H-BSE and L-BSE). Here, we report the results of our analyses of an H-BSE case found in Canada in 2021, indicating restriction of the pathological agent (PrPSc) mainly to the central nervous system with no or occasional weak involvement of peripheral tissues. Importantly, a non-synonymous mutation at codon 211 of the PRNP gene was detected and confirmed to be present as a germline mutation. This is the first case of BSE in Canada with a predisposing E211K mutation.
Snip…
Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.
Supplemental material Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene
KEYWORDS:
Atypical BSE Bovine Spongiform Encephalopathycentral nervous systemE211K mutationprion diseasesprion protein genesynonymous mutation
https://www.tandfonline.com/doi/full/10.1080/19336896.2025.2511933#d1e1606
“Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.”
MONDAY, JUNE 09, 2025
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE) The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE)
Component 6: Transmissible Spongiform Encephalopathies (TSEs)
Problem Statement 6A: Determine pathobiology of prion strains.
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE).
Virus and Prion Research Unit, National Animal Disease Center, Ames, Iowa
Classical BSE (C-BSE) is a prion disease of cattle that was responsible for the "mad cow disease" epizootic in Europe in the 1980s. C-BSE was determined to cause the human prion disease vCJD. Since then, atypical spontaneous strains of BSE were identified. H-BSE is one of those strains. Much research has explored the origins of C-BSE, and strain emergence from atypical H-BSE is one hypothesis. An H-BSE case was determined to have a germline mutation, an E211K substitution in the prion protein gene, which is analogous to a hereditary human prion disease. ARS scientists in Ames, Iowa reported the transmission of H-BSE from cattle, with and without the germline prion protein amino acid substitution, to cattle with various prion genotypes: EE211 (wild-type), EK211, and KK211. Results indicated a significantly shorter incubation period in K containing cattle compared to prion wild-type cattle. The scientists also explored the possibility that the C-BSE strain might have occurred after serial passages of EK211 and KK211 containing H-BSE in cattle, but results did not support this concept. This information is important to prion researchers, veterinary diagnostic laboratories, and those involved with establishing regulatory guidelines.
https://www.ars.usda.gov/ARSUserFiles/np103/AnnualReports/Final%20NP103%20FY2024%20Annual%20Report.updated%205.30.25.pdf
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research
Title: Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation
Author item Greenlee, Justin item Cassmann, Eric item MOORE, SARA JO - Oak Ridge Institute For Science And Education (ORISE) item WEST GREENLEE, HEATHER - Iowa State University
Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 6/24/2022 Publication Date: 9/16/2022 Citation: Greenlee, J.J., Cassmann, E.D., Moore, S., West Greenlee, H.M. 2022.
Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation.
Prion 2022 Conference abstracts: pushing the boundaries. 16(1):150. https://doi.org/10.1080/19336896.2022.2091286.
DOI: https://doi.org/10.1080/19336896.2022.2091286
Interpretive Summary:
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure. Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n=3) or from the US 2006 case with the E211K polymorphism (n=4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation. Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57-4.0) in the brainstem, and IHC demonstrated PrPSc throughout the brain. All cattle in the EE211 recipient group remain asymptomatic for the duration of the experiment (approximately 7 years post-inoculation).
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351
Highlights
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351
Title: A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation
Author item MOORE, S - Orise Fellow item WEST GREENLEE, M - Iowa State University item Smith, Jodi item Vrentas, Catherine item Nicholson, Eric item Greenlee, Justin
Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 8/30/2016 Publication Date: 9/15/2016 Citation: Moore, S.J., West Greenlee, M.H., Smith, J.D., Vrentas, C.E., Nicholson, E.M., Greenlee, J.J. 2016.
A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation.
Frontiers in Veterinary Science. 3:78. Interpretive
Summary: Cases of bovine spongiform encephalopathy (BSE) or mad cow disease can be subclassified into at least 3 distinct disease forms with the predominate form known as classical BSE and the others collectively referred to as atypical BSE. Atypical BSE can be further subdivided into H-type and L-type cases that are distinct from classical BSE and from each other. Both of the atypical BSE subtypes are believed to occur spontaneously, whereas classical BSE is spread through feeding contaminated meat and bone meal to cattle. Work by other research groups suggests that the stability of the distinguishing features of atypical BSE cases (phenotypical stability) can change to closely resemble classical BSE after experimental passage implicating atypical BSE as a possible origin of classical BSE. Interestingly, one case of H-type BSE in the US was associated with an inherited mutation in the prion protein gene referred to as E211K. The purpose of this work was to compare wild type and cattle with the E211K mutation after experimental inoculation with either classical BSE or H-BSE from the original E211K case. This study demonstrates that the disease features of E211K BSE-H remain stable when transmitted to cattle without the K211 polymorphism. In addition, passage of classical BSE to cattle with the K211 polymorphism results in disease with features consistent with classical BSE and not a switch to atypical BSE-H as a result of the K211 polymorphism. As the origin of classical, feedborne BSE remains unknown and low numbers of atypical BSE are diagnosed each year, parties with interest in the cattle and beef industries and regulatory officials responsible for safe feeding practices of cattle will be interested in this work.
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (BSE-H) was diagnosed in a cow that was associated with a heritable polymorphism in the bovine prion protein gene (PRNP) resulting in a lysine for glutamine amino acid substitution at codon 211 (called E211K) of the prion protein. Although the prevalence of this polymorphism is low, cattle carrying the K211 allele may be predisposed to rapid onset of BSE-H when exposed or to the potential development of a genetic BSE. This study was conducted to better understand the relationship between the K211 polymorphism and its effect on BSE phenotype. BSE-H from the US 2006 case was inoculated intracranially (IC) in one PRNP wild type (EE211) calf and one EK211 calf. In addition, one wild type calf and one EK211 calf were inoculated IC with brain homogenate from a US 2003 classical BSE case. All cattle developed clinical disease. The survival times of the E211K BSE-H inoculated EK211 calf (10 months) was shorter than the wild type calf (18 months). This genotype effect was not observed in classical BSE inoculated cattle (both 26 months). Significant changes in retinal function were observed in H-type BSE challenged cattle only. Cattle challenged with the same inoculum showed similar severity and neuroanatomical distribution of vacuolation and disease-associated prion protein deposition in the brain, though differences in neuropathology were observed between E211K BSE-H and classical BSE inoculated animals. Western blot results for brain tissue from challenged animals were consistent with the inoculum strains. This study demonstrates that the phenotype of E211K BSE-H remains stable when transmitted to cattle without the K211 polymorphism, and exhibits a number of features that differ from classical BSE in both wild type and heterozygous EK211 animals.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=326785
Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle
Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, * Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada. *Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca
Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.
Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.
Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.
Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.
Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.
Presentation Type: Oral Presentation Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute
Grant Number: ALMA/APRI: 201400006, HC 414250
Acknowledgement: TSE unit NCAD, Lethbridge (Jianmin Yang, Sarah Bogart, Rachana Muley, Yuanmu Fang, Keri Colwell, Renee Anderson, John Gray, Rakhi Katoch) (CFIA, Canada), Dr. Catherine Graham (NSDA, Canada), Dr. Michel Levy (UCVM, Canada), Dr. Martin Groschup (FLI, Germany), Dr. Christine Fast (FLI, Germany), Dr. Bob Hills (Health Canada, Canada) Theme: Animal prion diseases
"After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE. "
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PRION 2023 CONTINUED;
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https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
PLEASE NOTE, USDA ET AL ONLY TESTING <25k CATTLE FOR MAD COW DISEASE, woefully inadequate, yet USDA just documented a case Atypical L-Type BSE, the most virulent strain to date...
Wednesday, May 24, 2023
***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification
https://wahis.woah.org/#/in-review/5067
https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html
https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification
SATURDAY, MAY 20, 2023
***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE
https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html
https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed
MAY 19, 2023
https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse
ATYPICAL CASE OF BSE DETECTED IN SOUTH CAROLINA
May 19, 2023 By Meghan Grebner Filed Under: Beef, Human Interest, Livestock, News
BeefPacker.jpg The U.S. has reported an atypical case of Bovine Spongiform Encephalopathy (BSE) in a beef cow approximately five years old or older at a slaughter plant in South Carolina. The animal never entered slaughter channels and at no time presented a risk to the food supply or to human health in the U.S. The radio frequency identification tag present on the animal is associated with a herd in Tennessee. APHIS and veterinary officials in South Carolina and Tennessee are gathering more information during this ongoing investigation.
This is the nation’s 7th detection of BSE, of the six previous U.S. cases, the first, in 2003 was the only case of classical BSE, which was from a cow imported from Canada. The rest of the cases have been atypical BSE. The animal was tested as part of the USDA’s Animal and Plant Health Inspection Service’s routine surveillance of cattle that are deemed unsuitable for slaughter.
National Cattlemen’s Beef Association chief veterinarian Dr. Kathy Simmons says USDA’s ongoing BSE surveillance program has tested more than one million cattle since the program began, ensuring that the agency’s interlocking supply chain safety products are working. She says the incidence of BSE in the U.S. is extremely low and will remain so.
U.S. Cattlemen’s Association president Justin Tupper says the swift detection of this case proves that the systems and protocols put in place are working. He says the organization is grateful to the nationwide team of veterinarians, animal health officials, meat inspectors, and others who ensure the well-being of the U.S. cattle herd.
Atypical BSE generally occurs in older cattle and seems to arise rarely and spontaneously in all cattle populations.
The World Organization for Animal Health (WOAH) recognizes the U.S. as negligible risk for BSE, the lowest possible risk in the world. Per WOAH guidelines in determining this status, atypical BSE cases do not impact official BSE risk status and this finding of an atypical case will not change the negligible risk status of the U.S., and should not lead to any trade issues.
https://www.brownfieldagnews.com/news/atypical-case-of-bse-detected-in-south-carolina/
https://usbiotechnologyregulation.mrp.usda.gov/wcm/connect/aphis_content_library/sa_newsroom/sa_stakeholders/sa_by_date/sa-2023/bse
2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;
***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;
Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023
''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...
https://www.regulations.gov/comment/APHIS-2023-0027-0002
https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf
Saturday, April 25, 2026
USDA Statement BSE Surveillance Information Center Update 2026
https://oieusdabseprp.blogspot.com/2026/04/usda-statement-bse-surveillance.html
Mad cow disease: Could it be here? 2001 revisited 2026
https://prpsc.proboards.com/thread/230/mad-disease-2001-revisited-2026
https://bovineprp.blogspot.com/2026/08/mad-cow-disease-could-it-be-here-2001.html
TUESDAY, APRIL 07, 2026
APHIS USDA Captive CWD Herds Update by State March 2026
https://chronic-wasting-disease.blogspot.com/2026/04/aphis-usda-captive-cwd-herds-update-by.html
WEDNESDAY, APRIL 22, 2026
Atypical Bovine Spongiform Encephalopathy, Should we be Worried? Hong Kong Government
https://bovineprp.blogspot.com/2026/04/atypical-bovine-spongiform.html
https://prpsc.proboards.com/thread/214/atypical-bovine-spongiform-encephalopathy
Mortality of Individuals With PRNP Variants Associated With Prion Disease in the United States, 1998–2024 Singeltary Review 2026 August 26, 2026
what if ???
https://creutzfeldt-jakob-disease.blogspot.com/2026/08/mortality-of-individuals-with-prnp.html
https://prpsc.proboards.com/thread/237/mortality-variants-associated-prion-disease
WEDNESDAY, SEPTEMBER 2, 2026
A new chapter for CWRU’s Prion Center
https://prionunitusaupdate.blogspot.com/2026/09/a-new-chapter-for-cwrus-prion-center.html
https://prpsc.proboards.com/thread/239/new-chapter-cwru-prion-center
Terry S. Singeltary Sr.
Studies on atypical scrapie were identified as a key element of this block, given the potential risk associated to this agent. We studied the permeability of Human, bovine and porcine species barriers to atypical scrapie agent transmission. Experiments in transgenic mice expressing bovine, porcine or human PrPC suggest that this TSE agent has the intrinsic ability to propagate across these species barriers including the Human one. Upon species barrier passage the biological properties and phenotype of atypical scrapie seem to be altered. Further experiments are currently ongoing (in the framework of this project but also in other projects) in order to: (i) characterize the properties of the prion that emerged from the propagation of atypical scrapie in tg Hu; (ii) to confirm that the phenomena we observed are also true for atypical scrapie isolates other than the ones we have studied.
In parallel, studies in sheep have concluded that:
*** Atypical scrapie can be transmitted by both oral and intracerebral route in sheep with various PRP genotypes
*** Low but consistent amount of infectivity accumulates in peripheral tissue (mammary gland, lymph nodes, placenta, skeletal muscles, nerves) of sheep incubating atypical scrapie.
*** The combination of data from all our studies leads us to conclude that:
*** Atypical scrapie passage through species barriers can lead to the emergence of various prions including classical BSE (following propagation in porcine PRP transgenic mice).
*** Atypical scrapie can propagate, with a low efficacy, in human PrP expressing mice. This suggests the existence of a zoonotic potential for this TSE agent.
snip...
We advance our main conclusions and recommendations, in particular as they might affect public policy, including a detailed elaboration of the evidence that led to them. Our main recommendations are:
a. The issue of re-introducing ruminant protein into the food-chain The opinion of the members of Priority is that sustaining an absolute feed ban for ruminant protein to ruminants is the essential requirement, especially since the impact of non-classical forms of scrapie in sheep and goats is not fully understood and cannot be fully estimated. Therefore, the consortium strongly recommends prohibiting re-introduction of processed ruminant protein into the food-chain. Arguments in support of this opinion are:
• the large (and still uncharacterized) diversity of prion agents that circulate in animal populations;
• the uncertainties related to prion epidemiology in animal populations;
• the unknown efficacy of industrial processes applied to reduce microbiological risk during processed animal protein (PAP) production on most prion agents; • the intrinsic capacity of prions to cross interspecies transmission barriers; • the lack of sensitive methodology for identifying cross contamination in food.
• the evolution of natural food chains in nature (i.e. who eats whom or what) has generated an efficient barrier preventing, to some extent, novel prion epidemies and that this naturally evolved ecology should be respected.
The consortium is also hesitant to introduce processed ruminant proteins into fish food considering the paucity of data on prion infections in fishes and sea animals including those of mammalian origin, and the risk of establishing an environmental contamination of the oceans that cannot be controlled.
b. Atypical prion agents and surveillance
Atypical prion agents (see below) will probably continue to represent the dominant form of prion diseases in the near future, particularly in Europe.
*** Atypical L-type BSE has clear zoonotic potential, as demonstrated in experimental models.
*** Similarly, there are now some data that seem to indicate that the atypical scrapie agent can cross various species barriers.
*** Moreover, the current EU policy for eradicating scrapie (genetic selection in affected flocks) is ineffective for preventing atypical scrapie.
*** The recent identification of cell-to-cell propagation and the protein-encoded strain properties of human neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, suggest that they bear the potential to be transmissible even if not with the same efficiency as CJD. More epidemiological data from large cohorts are necessary to reach any conclusion on the impact of their transmissibility on public health. Re-evaluations of safety precautions may become necessary depending on the outcome of these studies. In that context it would appear valuable
• to develop knowledge related to the pathogenesis and inter-individual transmission of atypical prion agents in ruminants (both intra- and inter-species)
• to improve the sensitivity of detection assays that are applied in the field towards this type of agent
• to maintain a robust surveillance of both animal and human populations
c. The need for extended research on prions
Intensified searching for a molecular determinants of the species barrier is recommended, since this barrier is a key for many important policy areas - risk assessment, proportional policies, the need for screening of human products and food. In this respect, prion strain structural language also remains an important issue for public health for the foreseeable future. Understanding the structural basis for strains and the basis for adaptation of a strain to a new host will require continued fundamental research. Prions maintain a complex two-way relationship with the host cell and fundamental research is needed on mechanisms for their transmission, replication and cause of nervous system dysfunction and death.
Early detection of prion infection, ideally at preclinical stage, also remains crucial for development of effective treatment strategies in humans affected by the disease.
Position of the Priority consortium
Nearly 30 years ago, the appearance in the UK of Bovine Spongiform Encephalopathy (BSE) quickly brought the previously obscure “prion diseases” to the spotlight. The ensuing health and food crises that spread throughout Europe had devastating consequences. In the UK alone, there were more than 36,000 farms directly affected by BSE and the transmission of BSE prions to humans via the food chain has caused over 200 people in Europe to die from variant Creutzfeldt-Jakob disease (vCJD) (http://www.cjd.ed.ac.uk
Origins of prion epidemies
Classical BSE now appears to be under control, with 18 EU Member States having achieved the World Organisation for Animal Health (Office International Epizooties) „negligible risk‟ status (May 2014; http://www.oie.int/en/animal-health-in-the-world/official-disease-status/bse/list-of-bse-risk-status/), and the remaining MS assessed as „controlled‟ risk. Of note, research, including EU-funded research, has played a key role in this success: while the origin of the infection was never defined, the principle driver of the epidemic was identified as prions in Meat and Bone Meal (MBM). Tests based on prion protein-specific antibodies were developed, allowing detection of infected animals, and a better understanding of disease pathogenesis and the distribution of infectivity in edible tissues; experimental investigation of transmission barriers between different species allowed a rational estimation of risks, etc. All of this led to the implementation of rational and effective policies, such as the MBM ban to protect the animal feed chain, and the Specified Risk Material (SRM) regulations to protect the human food chain.
In spite of this progress, prions are still a threat. Epidemiological re-assessment indicates that the ∼10 year incubation period separating the peaks of the BSE and the vCJD epidemics is probably too short. In addition, results from a large number of human tonsil and appendix analyses in the UK suggest that there may be a high number of asymptomatic individuals who are positive for the disease-associated conformer prion protein PrPSc. While vCJD is the only form of human prion disease that has been consistently demonstrated to have lymphoreticular involvement, there has been no systematic investigation of lymphoid tissue in cases with other prion diseases.
The human prion problem
The clinical cases of vCJD identified to date have all shared a common PrP genotype (M129M), although one pre-clinical case was confirmed as an M129V heterozygote, and it has been mooted that perhaps only the M129M proportion of the population is susceptible. However, in the UK appendix study, PrP accumulation was described in samples representing every codon 129 genotype, raising the possibility that genotype does not confer resistance but instead modulates incubation period. Apart from the two UK studies, the lymphoid tissues of non-CJD patients have not been examined for the presence of PrPSc, so, these cases may not solely represent pre-clinical vCJD, but also other forms of prion disease.
Recent experiments in highly susceptible mouse models indicate the presence of infectivity in blood or blood components at late disease stages in sporadic CJD. The significance of this experimental finding for humans has to be explored in more detail and, at the present time, there is no evidence for the transmission of prions via blood in sporadic CJD. However a likely scenario is that all those with signs of infection or abnormal PrP accumulation in peripheral tissue could have infective blood, posing the risk for transmission via blood products, which has been clearly demonstrated in experimental models, and confirmed in several cases of vCJD in man. Altogether, these data clearly demonstrate the potential risk of a second wave of vCJD, particularly when the number people identified with lymphoid accumulation of PrPSc (16/32,411) gives a prevalence estimate in the UK of 493 per million, much higher than the number of clinical cases seen to date.
The animal prion problem
An increasing number of reports on cases of “atypical” BSE in cattle throughout the EU and beyond may lead to a new epidemic, particularly since we still do not understand all factors determining the species barrier. Ovine scrapie is another concern, because it could mask ovine BSE, presumably transmissible to humans. Scrapie is endemic and not likely to be eradicated soon, although current control measures are effective at greatly reducing disease incidence. Atypical forms, which may be spontaneous, are not affected by these control measures and these forms of disease will persist in the global animal population. The low prevalence of these disease forms makes effective surveillance very challenging. However, there is a clear risk attendant on ignoring these cases without an understanding of their possible zoonotic potential, particularly when most forms of human disease have no established aetiology. In summary, atypical cases of BSE and scrapie presently clearly outnumber classical cases in cattle and sheep in all member states.
We will highlight the state-of-the-art knowledge and point out scientific challenges and the major questions for research. Strategic objectives and priorities in Europe in the future for research that aims to control, eliminate or eradicate the threat posed by prions to our food and health are also indicated.
The Priority project has focused on 4 themes, namely the structure, function, conversion and toxicity of prions; detection of prions; mechanisms of prion transmission and spreading and epidemiology of prion diseases. This paper summarizes the opinions/positions reached within these themes at the end of the project.
http://cordis.europa.eu/docs/results/222/222887/final1-priority-final-report.pdf
see;
https://nor-98.blogspot.com/2016/09/goat-k222-prpc-polymorphic-variant-does.html
Transmission of scrapie prions to primate after an extended silent incubation period
*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.
*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160
O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations
*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases.
==============
PRION 2015 CONFERENCE
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5019500/
PRION 2016 TOKYO
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-68961933-690X
WS-01: Prion diseases in animals and zoonotic potential
***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.
***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.
***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20
Title: Transmission of scrapie prions to primate after an extended silent incubation period)
*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.
*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160
Comparing the Distribution of Ovine Classical Scrapie and Sporadic Creutzfeldt-Jakob Disease in Italy: Spatial and Temporal Associations (2002-2014)
Ru G1 ., Pocchiari M2 ., Bertolini S. 1, Pite L.1 , Puopolo M.2 , Ladogana A.2 , Perrotta M.G.3 , Meloni D 1 . (1) National reference center for the study and research on animal encephalopathies and comparative neuropathologies (CEA). Experimental Zooprophylactic Institute of Piemonte, Liguria and Valle d'Aosta, Torino, Italy.
(2) Department of Cellular Biology and Neuroscience, Istituto Superiore di Sanità, Roma, Italy. (3) Office 3 National center for the fight and emergency against animal diseases. Ministry of Health, Roma, Italy.
Aim: This study aims to investigate potential spatial and temporal associations between Creutzfeldt-Jakob disease (CJD) in humans (2010-2014) and ovine classical scrapie (CS) (2002- 2006) in Italy, serving as a proxy for exposure.
Materials and Methods: National data from prion disease surveillance in humans (sporadic CJD) and small ruminants (CS) in Italy were utilized. A descriptive geographic analysis was conducted for each disease individually. Subsequently, an ecological study was performed to compare the occurrence of both diseases at the district and regional levels. Standardized incidence ratios (SIR), adjusted for confounders, were calculated for CJD and CS by district and region, respectively, representing the outcome and proxy of exposure. Considering a possible long incubation period of CJD, two study periods were analysed: 2010-2014 for CJD and 2002-2006 for CS. Eight alternative linear regression models were developed using SIR in humans as the dependent variable and SIR in sheep as the independent variable. These models varied in the scale of SIR data (continuous vs. categorical), geographical level (district vs. region), and the potential past exposure of sheep in specific areas to a known source of infection (via a contaminated vaccine).
Results: The analysis of data at the district level revealed no significant association. However, when considering aggregated regional data, all four models consistently indicated a statistically significant positive association, suggesting a higher incidence of the disease in humans as the regional incidence of sheep scrapie increased.
Conclusions: While the results are intriguing, it is important to acknowledge the inherent limitations of ecological studies. Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.
Funded by: Italian Ministry of Health Grant number: Realizzazione del programma epidemiologico finalizzato a dare evidenza del potenziale zoonotico delle TSE animali diverse dalla BSE. Prot. N. 0018730-17/07/2015-DGSAFCOD_UO-P
''Nevertheless, these findings provide valuable evidence to formulate a hypothesis regarding the zoonotic potential of classical scrapie. Further investigations are necessary, employing specific designs such as analytical epidemiology studies, to test this hypothesis effectively.''
Meeting-book-final-version prion 2023 Prion 2023 Congress Organizing Committee and the NeuroPrion Association, we invite you to join us for the International Conference Prion2023 from 16-20 October 2023 in Faro, Portugal.
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene novel E211K polymorphism in prion protein gene
Waqas Tahir , Sandor Dudas , Renee Anderson , Jianmin Yang , Sarah Bogart , Kristina Santiago-Mateo, Yuanmu Fang & Roberta Quaghebeur
Pages 36-49 | Received 20 Feb 2025, Accepted 22 May 2025, Published online: 04 Aug 2025 Cite this article https://doi.org/10.1080/19336896.2025.2511933
ABSTRACT
Bovine Spongiform Encephalopathy (BSE) is a fatal neurodegenerative disease in cattle which can be either classical BSE (C-BSE) or atypical BSE (including H-BSE and L-BSE). Here, we report the results of our analyses of an H-BSE case found in Canada in 2021, indicating restriction of the pathological agent (PrPSc) mainly to the central nervous system with no or occasional weak involvement of peripheral tissues. Importantly, a non-synonymous mutation at codon 211 of the PRNP gene was detected and confirmed to be present as a germline mutation. This is the first case of BSE in Canada with a predisposing E211K mutation.
Snip…
Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.
Supplemental material Canadian 2021 H-type Bovine Spongiform Encephalopathy case associated with a novel E211K polymorphism in prion protein gene
KEYWORDS:
Atypical BSE Bovine Spongiform Encephalopathycentral nervous systemE211K mutationprion diseasesprion protein genesynonymous mutation
https://www.tandfonline.com/doi/full/10.1080/19336896.2025.2511933#d1e1606
“Based on the results of this study, and the 2006 H-BSE case in the USA, there is an expanded spectrum of aetiologies for bovine prion diseases similar to what is observed in humans, including sporadic, genetic and acquired versions.”
MONDAY, JUNE 09, 2025
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE) The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE)
Component 6: Transmissible Spongiform Encephalopathies (TSEs)
Problem Statement 6A: Determine pathobiology of prion strains.
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE).
Virus and Prion Research Unit, National Animal Disease Center, Ames, Iowa
Classical BSE (C-BSE) is a prion disease of cattle that was responsible for the "mad cow disease" epizootic in Europe in the 1980s. C-BSE was determined to cause the human prion disease vCJD. Since then, atypical spontaneous strains of BSE were identified. H-BSE is one of those strains. Much research has explored the origins of C-BSE, and strain emergence from atypical H-BSE is one hypothesis. An H-BSE case was determined to have a germline mutation, an E211K substitution in the prion protein gene, which is analogous to a hereditary human prion disease. ARS scientists in Ames, Iowa reported the transmission of H-BSE from cattle, with and without the germline prion protein amino acid substitution, to cattle with various prion genotypes: EE211 (wild-type), EK211, and KK211. Results indicated a significantly shorter incubation period in K containing cattle compared to prion wild-type cattle. The scientists also explored the possibility that the C-BSE strain might have occurred after serial passages of EK211 and KK211 containing H-BSE in cattle, but results did not support this concept. This information is important to prion researchers, veterinary diagnostic laboratories, and those involved with establishing regulatory guidelines.
https://www.ars.usda.gov/ARSUserFiles/np103/AnnualReports/Final%20NP103%20FY2024%20Annual%20Report.updated%205.30.25.pdf
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research
Title: Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation
Author item Greenlee, Justin item Cassmann, Eric item MOORE, SARA JO - Oak Ridge Institute For Science And Education (ORISE) item WEST GREENLEE, HEATHER - Iowa State University
Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 6/24/2022 Publication Date: 9/16/2022 Citation: Greenlee, J.J., Cassmann, E.D., Moore, S., West Greenlee, H.M. 2022.
Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation.
Prion 2022 Conference abstracts: pushing the boundaries. 16(1):150. https://doi.org/10.1080/19336896.2022.2091286.
DOI: https://doi.org/10.1080/19336896.2022.2091286
Interpretive Summary:
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure. Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n=3) or from the US 2006 case with the E211K polymorphism (n=4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation. Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57-4.0) in the brainstem, and IHC demonstrated PrPSc throughout the brain. All cattle in the EE211 recipient group remain asymptomatic for the duration of the experiment (approximately 7 years post-inoculation).
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351
Highlights
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=395351
Title: A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation
Author item MOORE, S - Orise Fellow item WEST GREENLEE, M - Iowa State University item Smith, Jodi item Vrentas, Catherine item Nicholson, Eric item Greenlee, Justin
Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 8/30/2016 Publication Date: 9/15/2016 Citation: Moore, S.J., West Greenlee, M.H., Smith, J.D., Vrentas, C.E., Nicholson, E.M., Greenlee, J.J. 2016.
A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation.
Frontiers in Veterinary Science. 3:78. Interpretive
Summary: Cases of bovine spongiform encephalopathy (BSE) or mad cow disease can be subclassified into at least 3 distinct disease forms with the predominate form known as classical BSE and the others collectively referred to as atypical BSE. Atypical BSE can be further subdivided into H-type and L-type cases that are distinct from classical BSE and from each other. Both of the atypical BSE subtypes are believed to occur spontaneously, whereas classical BSE is spread through feeding contaminated meat and bone meal to cattle. Work by other research groups suggests that the stability of the distinguishing features of atypical BSE cases (phenotypical stability) can change to closely resemble classical BSE after experimental passage implicating atypical BSE as a possible origin of classical BSE. Interestingly, one case of H-type BSE in the US was associated with an inherited mutation in the prion protein gene referred to as E211K. The purpose of this work was to compare wild type and cattle with the E211K mutation after experimental inoculation with either classical BSE or H-BSE from the original E211K case. This study demonstrates that the disease features of E211K BSE-H remain stable when transmitted to cattle without the K211 polymorphism. In addition, passage of classical BSE to cattle with the K211 polymorphism results in disease with features consistent with classical BSE and not a switch to atypical BSE-H as a result of the K211 polymorphism. As the origin of classical, feedborne BSE remains unknown and low numbers of atypical BSE are diagnosed each year, parties with interest in the cattle and beef industries and regulatory officials responsible for safe feeding practices of cattle will be interested in this work.
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (BSE-H) was diagnosed in a cow that was associated with a heritable polymorphism in the bovine prion protein gene (PRNP) resulting in a lysine for glutamine amino acid substitution at codon 211 (called E211K) of the prion protein. Although the prevalence of this polymorphism is low, cattle carrying the K211 allele may be predisposed to rapid onset of BSE-H when exposed or to the potential development of a genetic BSE. This study was conducted to better understand the relationship between the K211 polymorphism and its effect on BSE phenotype. BSE-H from the US 2006 case was inoculated intracranially (IC) in one PRNP wild type (EE211) calf and one EK211 calf. In addition, one wild type calf and one EK211 calf were inoculated IC with brain homogenate from a US 2003 classical BSE case. All cattle developed clinical disease. The survival times of the E211K BSE-H inoculated EK211 calf (10 months) was shorter than the wild type calf (18 months). This genotype effect was not observed in classical BSE inoculated cattle (both 26 months). Significant changes in retinal function were observed in H-type BSE challenged cattle only. Cattle challenged with the same inoculum showed similar severity and neuroanatomical distribution of vacuolation and disease-associated prion protein deposition in the brain, though differences in neuropathology were observed between E211K BSE-H and classical BSE inoculated animals. Western blot results for brain tissue from challenged animals were consistent with the inoculum strains. This study demonstrates that the phenotype of E211K BSE-H remains stable when transmitted to cattle without the K211 polymorphism, and exhibits a number of features that differ from classical BSE in both wild type and heterozygous EK211 animals.
https://www.ars.usda.gov/research/publications/publication/?seqNo115=326785
Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle
Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, * Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada. *Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca
Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.
Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.
Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.
Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.
Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.
Presentation Type: Oral Presentation Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute
Grant Number: ALMA/APRI: 201400006, HC 414250
Acknowledgement: TSE unit NCAD, Lethbridge (Jianmin Yang, Sarah Bogart, Rachana Muley, Yuanmu Fang, Keri Colwell, Renee Anderson, John Gray, Rakhi Katoch) (CFIA, Canada), Dr. Catherine Graham (NSDA, Canada), Dr. Michel Levy (UCVM, Canada), Dr. Martin Groschup (FLI, Germany), Dr. Christine Fast (FLI, Germany), Dr. Bob Hills (Health Canada, Canada) Theme: Animal prion diseases
"After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE. "
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PRION 2023 CONTINUED;
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
PLEASE NOTE, USDA ET AL ONLY TESTING <25k CATTLE FOR MAD COW DISEASE, woefully inadequate, yet USDA just documented a case Atypical L-Type BSE, the most virulent strain to date...
Wednesday, May 24, 2023
***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification
https://wahis.woah.org/#/in-review/5067
https://woahoie.blogspot.com/2023/05/wahis-woah-oie-united-states-of-america.html
https://prpsc.proboards.com/thread/125/wahis-woah-oie-immediate-notification
SATURDAY, MAY 20, 2023
***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE
https://bse-atypical.blogspot.com/2023/05/tennessee-state-veterinarian-alerts.html
https://prpsc.proboards.com/thread/123/tennessee-veterinarian-alerts-cattle-confirmed
MAY 19, 2023
https://www.aphis.usda.gov/aphis/newsroom/stakeholder-info/sa_by_date/sa-2023/bse
ATYPICAL CASE OF BSE DETECTED IN SOUTH CAROLINA
May 19, 2023 By Meghan Grebner Filed Under: Beef, Human Interest, Livestock, News
BeefPacker.jpg The U.S. has reported an atypical case of Bovine Spongiform Encephalopathy (BSE) in a beef cow approximately five years old or older at a slaughter plant in South Carolina. The animal never entered slaughter channels and at no time presented a risk to the food supply or to human health in the U.S. The radio frequency identification tag present on the animal is associated with a herd in Tennessee. APHIS and veterinary officials in South Carolina and Tennessee are gathering more information during this ongoing investigation.
This is the nation’s 7th detection of BSE, of the six previous U.S. cases, the first, in 2003 was the only case of classical BSE, which was from a cow imported from Canada. The rest of the cases have been atypical BSE. The animal was tested as part of the USDA’s Animal and Plant Health Inspection Service’s routine surveillance of cattle that are deemed unsuitable for slaughter.
National Cattlemen’s Beef Association chief veterinarian Dr. Kathy Simmons says USDA’s ongoing BSE surveillance program has tested more than one million cattle since the program began, ensuring that the agency’s interlocking supply chain safety products are working. She says the incidence of BSE in the U.S. is extremely low and will remain so.
U.S. Cattlemen’s Association president Justin Tupper says the swift detection of this case proves that the systems and protocols put in place are working. He says the organization is grateful to the nationwide team of veterinarians, animal health officials, meat inspectors, and others who ensure the well-being of the U.S. cattle herd.
Atypical BSE generally occurs in older cattle and seems to arise rarely and spontaneously in all cattle populations.
The World Organization for Animal Health (WOAH) recognizes the U.S. as negligible risk for BSE, the lowest possible risk in the world. Per WOAH guidelines in determining this status, atypical BSE cases do not impact official BSE risk status and this finding of an atypical case will not change the negligible risk status of the U.S., and should not lead to any trade issues.
https://www.brownfieldagnews.com/news/atypical-case-of-bse-detected-in-south-carolina/
https://usbiotechnologyregulation.mrp.usda.gov/wcm/connect/aphis_content_library/sa_newsroom/sa_stakeholders/sa_by_date/sa-2023/bse
2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;
***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;
Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023
''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...
https://www.regulations.gov/comment/APHIS-2023-0027-0002
https://downloads.regulations.gov/APHIS-2023-0027-0002/attachment_1.pdf
Saturday, April 25, 2026
USDA Statement BSE Surveillance Information Center Update 2026
https://oieusdabseprp.blogspot.com/2026/04/usda-statement-bse-surveillance.html
Mad cow disease: Could it be here? 2001 revisited 2026
https://prpsc.proboards.com/thread/230/mad-disease-2001-revisited-2026
https://bovineprp.blogspot.com/2026/08/mad-cow-disease-could-it-be-here-2001.html
TUESDAY, APRIL 07, 2026
APHIS USDA Captive CWD Herds Update by State March 2026
https://chronic-wasting-disease.blogspot.com/2026/04/aphis-usda-captive-cwd-herds-update-by.html
WEDNESDAY, APRIL 22, 2026
Atypical Bovine Spongiform Encephalopathy, Should we be Worried? Hong Kong Government
https://bovineprp.blogspot.com/2026/04/atypical-bovine-spongiform.html
https://prpsc.proboards.com/thread/214/atypical-bovine-spongiform-encephalopathy
Mortality of Individuals With PRNP Variants Associated With Prion Disease in the United States, 1998–2024 Singeltary Review 2026 August 26, 2026
what if ???
https://creutzfeldt-jakob-disease.blogspot.com/2026/08/mortality-of-individuals-with-prnp.html
https://prpsc.proboards.com/thread/237/mortality-variants-associated-prion-disease
WEDNESDAY, SEPTEMBER 2, 2026
A new chapter for CWRU’s Prion Center
https://prionunitusaupdate.blogspot.com/2026/09/a-new-chapter-for-cwrus-prion-center.html
https://prpsc.proboards.com/thread/239/new-chapter-cwru-prion-center
Terry S. Singeltary Sr.
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