Sunday, January 15, 2017

US lifts French beef MAD COW BSE import embargo, France says… LOL!

US lifts French beef MAD COW BSE import embargo, France says… LOL!

January 14, 2017

US lifts French beef import embargo, France says

US authorities have lifted an embargo on French beef imports after 19 years, the French agriculture ministry said Friday.

France is the fourth EU country to have its beef re-admitted to the US market after a 1998 ban imposed because of fears over bovine spongiform encephalopathy (BSE), also known as mad cow disease.

The others are Ireland, Lithuania and the Netherlands.

The EU Commission welcomed the move, calling it "excellent news for French producers". It was also an illustration that efforts to eradicate BSE in the EU had borne fruit, it said in a statement.

The French ministry warned, however, that administrative hurdles meant it could take time for beef exports to the US to resume.

"We are pleased with this first step, but this doesn't mean that exports will start tomorrow," the ministry said.


>>> US lifts French beef import embargo, France says The EU Commission welcomed the move, calling it "excellent news for French producers". It was also an illustration that efforts to eradicate BSE in the EU had borne fruit, it said in a statement. <<< 

LOL! THE MAD COW FOLLIES CONTINUE, THANKS TO THE USDA AND THE OIE $$$

FRANCE stopped testing for mad cow disease because of an unusual outbreak of atypical MAD COW DISEASE.

THE OIE BSE MRR POLICY IS NOTHING MORE THAN A LEGAL TOOL TO TRADE TSE PRION AKA MAD COW TYPE DISEASE GLOBALLY, IT'S ALL ABOUT MONEY AND TRADE HUMAN LIFE IS HINDSIGHT OR AN AFTER THOUGHT $$$ 

Thursday, March 24, 2016 

FRANCE CONFIRMS BOVINE SPONGIFORM ENCEPHALOPATHY BSE MAD COW (ESB) chez une vache dans les Ardennes 


MONDAY, MAY 2, 2016 

France Confirms Case of Classical Mad Cow Disease BSE

BSE identified in France


*** France stops BSE testing for Mad Cow Disease


***atypical spontaneous BSE in France LOL***

SPONTANEOUS ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY

***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.***


FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many spontaneous events of mad cow disease $$$

spontaneous atypical BSE ???

if that's the case, then France is having one hell of an epidemic of atypical BSE, probably why they stopped testing for BSE, problem solved $$$

As of December 2011, around 60 atypical BSE cases have currently been reported in 13 countries, *** with over one third in France.

 http://www.biomedcentral.com/1746-6148/8/74

so 20 cases of atypical BSE in France, compared to the remaining 40 cases in the remaining 12 Countries, divided by the remaining 12 Countries, about 3+ cases per country, besides Frances 20 cases. you cannot explain this away with any spontaneous BSe. ...TSS

If you Compare France to other Countries with atypical BSE, in my opinion, you cannot explain this with ‘spontaneous’.

Table 1: Number of Atypical BSE cases reported by EU Member States in the period 2001–2014 by country and by type (L- and H-BSE) (extracted from EU BSE databases on 1 July 2014). By 2015, these data might be more comprehensive following a request from the European Commission to Member States for re-testing and retrospective classification of all positive bovine isolates in the EU in the years 2003–2009

BSE type

Country 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013(a) 2014(a) Total

H-BSE Austria 1 1

France(b) 1 2 3 1 2 2 2 2 15

Germany 1 1 2

Ireland 1 1 2 1 5

The Netherlands 1 1

Poland 1 1 2

Portugal 1 1

Spain 1 1 2

Sweden 1 1

United Kingdom 1 1 1 1 1 5

Total 2 3 3 1 1 2 2 2 4 4 5 1 4 1 35

L-BSE Austria 1 1 2

Denmark 1 1

*** France(b) 1 1 1 1 2 1 3 2 1 1 14

Germany 1 1 2

Italy 1 1 1 1 1 5

The Netherlands 1 1 1 3

Poland 1 2 2 1 2 1 2 1 12

Spain 2 2

United Kingdom 1 1 1 1 4

Total 0 5 3 4 3 3 6 3 3 4 3 6 1 1 45

Total Atypical cases (H + L)

2 8 6 5 4 5 8 5 7 8 8 7 5 2 80

(a): Data for 2013-2014 are incomplete and may not include all cases/countries reported.

(b): France has performed extensive retrospective testing to classify BSE cases, which is probably the explanation for the higher number of Atypical BSE cases reported in this country.

The number of Atypical BSE cases detected in countries that have already identified them seems to be similar from year to year. In France, a retrospective study of all TSE-positive cattle identified through the compulsory EU surveillance between 2001 and 2007 indicated that the prevalence of H-BSE and L-BSE was 0.35 and 0.41 cases per million adult cattle tested, respectively, which increased to 1.9 and 1.7 cases per million, respectively, in tested animals over eight years old (Biacabe et al., 2008). No comprehensive study on the prevalence of Atypical BSE cases has yet been carried out in other EU Member States. All cases of Atypical BSE reported in the EU BSE databases have been identified by active surveillance testing (59 % in fallen stock, 38 % in healthy slaughtered cattle and 4 % in emergency slaughtered cattle). Cases were reported in animals over eight years of age, with the exception of two cases (one H-BSE and one L-BSE) detected in Spain in 2011/2012. One additional case of H-BSE was detected in Switzerland in 2012 in a cow born in Germany in 2005 (Guldimann et al., 2012).



Wednesday, July 15, 2015

Additional BSE TSE prion testing detects pathologic lesion in unusual brain location and PrPsc by PMCA only, how many cases have we missed?


***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67 PrPsc was not detected using rapid tests for BSE.

***Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.

*** IBNC Tauopathy or TSE Prion disease, it appears, no one is sure ***

Posted by Terry S. Singeltary Sr. on 03 Jul 2015 at 16:53 GMT


*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;


*** It also suggests a similar cause or source for atypical BSE in these countries. ***

Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan.

*** This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada.

*** It also suggests a similar cause or source for atypical BSE in these countries. ***

see page 176 of 201 pages...tss


Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

snip...

The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...


***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.

P.86: Estimating the risk of transmission of BSE and scrapie to ruminants and humans by protein misfolding cyclic amplification

Morikazu Imamura, Naoko Tabeta, Yoshifumi Iwamaru, and Yuichi Murayama

National Institute of Animal Health; Tsukuba, Japan

To assess the risk of the transmission of ruminant prions to ruminants and humans at the molecular level, we investigated the ability of abnormal prion protein (PrPSc) of typical and atypical BSEs (L-type and H-type) and typical scrapie to convert normal prion protein (PrPC) from bovine, ovine, and human to proteinase K-resistant PrPSc-like form (PrPres) using serial protein misfolding cyclic amplification (PMCA).

Six rounds of serial PMCA was performed using 10% brain homogenates from transgenic mice expressing bovine, ovine or human PrPC in combination with PrPSc seed from typical and atypical BSE- or typical scrapie-infected brain homogenates from native host species. In the conventional PMCA, the conversion of PrPC to PrPres was observed only when the species of PrPC source and PrPSc seed matched. However, in the PMCA with supplements (digitonin, synthetic polyA and heparin), both bovine and ovine PrPC were converted by PrPSc from all tested prion strains. On the other hand, human PrPC was converted by PrPSc from typical and H-type BSE in this PMCA condition.

Although these results were not compatible with the previous reports describing the lack of transmissibility of H-type BSE to ovine and human transgenic mice, our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.


P.170: Potential detection of oral transmission of H type atypical BSE in cattle using in vitro conversion

***P.170: Potential detection of oral transmission of H type atypical BSE in cattle using in vitro conversion

Sandor Dudas, John G Gray, Renee Clark, and Stefanie Czub Canadian Food Inspection Agency; Lethbridge, AB Canada

Keywords: Atypical BSE, oral transmission, RT-QuIC

The detection of bovine spongiform encephalopathy (BSE) has had a significant negative impact on the cattle industry worldwide. In response, governments took actions to prevent transmission and additional threats to animal health and food safety. While these measures seem to be effective for controlling classical BSE, the more recently discovered atypical BSE has presented a new challenge. To generate data for risk assessment and control measures, we have challenged cattle orally with atypical BSE to determine transmissibility and mis-folded prion (PrPSc) tissue distribution. Upon presentation of clinical symptoms, animals were euthanized and tested for characteristic histopathological changes as well as PrPSc deposition.

The H-type challenged animal displayed vacuolation exclusively in rostral brain areas but the L-type challenged animal showed no evidence thereof. To our surprise, neither of the animals euthanized, which were displaying clinical signs indicative of BSE, showed conclusive mis-folded prion accumulation in the brain or gut using standard molecular or immunohistochemical assays. To confirm presence or absence of prion infectivity, we employed an optimized real-time quaking induced conversion (RT-QuIC) assay developed at the Rocky Mountain Laboratory, Hamilton, USA.

Detection of PrPSc was unsuccessful for brain samples tests from the orally inoculated L type animal using the RT-QuIC. It is possible that these negative results were related to the tissue sampling locations or that type specific optimization is needed to detect PrPSc in this animal. We were however able to consistently detect the presence of mis-folded prions in the brain of the H-type inoculated animal. Considering the negative and inconclusive results with other PrPSc detection methods, positive results using the optimized RT-QuIC suggests the method is extremely sensitive for H-type BSE detection. This may be evidence of the first successful oral transmission of H type atypical BSE in cattle and additional investigation of samples from these animals are ongoing.




Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

snip...

The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...




In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells

3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. ...


The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite it’s subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA veiwed it as a wildlife problem and consequently not their province! ...page 26.


*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.



SPONTANEOUS ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY

***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.***


Primate Biol., 3, 47–50, 2016 www.primate-biol.net/3/47/2016/ doi:10.5194/pb-3-47-2016 © Author(s) 2016. CC

Attribution 3.0 License.

Prions

Walter Bodemer German Primate Center, Kellnerweg 4, 37077 Göttingen, Germany Correspondence to: Walter Bodemer (wbodemer@dpz.eu)

Received: 15 June 2016 – Revised: 24 August 2016 – Accepted: 30 August 2016 – Published: 7 September 2016

SNIP...
  
3 Conclusion

Most importantly, early signs of an altered circadian rhythm, sleep–wake cycle, and activity and body temperature were recorded in prion-infected animals. This experimental approach would have never been feasible in studies with human CJD cases. After 4–6 years animals developed clinical symptoms highly similar to those typical for CJD. Clinicians confirmed how close the animal model and the human disease matched. Non-neuronal tissue like cardiac muscle and peripheral blood with abnormal, disease-related prion protein were detected in rhesus monkey tissues. 

Molecular changes in RNA from repetitive Alu and BC200 DNA elements were identified and found to be targets of epigenetic editing mechanisms active in prion disease. To conclude, our results with the rhesus monkey model for prion disease proved to be a valid model and increased our knowledge of pathogenic processes that are distinctive to prion disease.

SEE FULL TEXT ;
  

O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations

 Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France

 Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.

 *** 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. We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.

 ===============

***thus questioning the origin of human sporadic cases***

 ***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. 
  

Saturday, April 23, 2016

PRION 2016 TOKYO

Saturday, April 23, 2016

 SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016

 Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online

Taylor & Francis

Prion 2016 Animal Prion Disease Workshop Abstracts

WS-01: Prion diseases in animals and zoonotic potential

Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a,

Natalia Fernandez-Borges a. and Alba Marin-Moreno a

"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. France

 Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion. Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier.

 To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents.

These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant.

 Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with ef?ciency comparable to that of cattle BSE. While the ef?ciency 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. 


why do we not want to do TSE transmission studies on chimpanzees $

5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

 snip...

 R. BRADLEY


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.


SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016

Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online



Tuesday, July 21, 2009

Transmissible mink encephalopathy - review of the etiology


Saturday, December 01, 2007

Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model


Sunday, December 10, 2006

Transmissible Mink Encephalopathy TME



Saturday, June 25, 2011

Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque

"BSE-L in North America may have existed for decades"


Wednesday, April 25, 2012

4th MAD COW DISEASE U.S.A. CALIFORNIA ATYPICAL L-TYPE BSE 2012


Thursday, October 22, 2015

Former Ag Secretary Ann Veneman talks women in agriculture and we talk mad cow disease USDA and what really happened


Sunday, December 15, 2013

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2013 UPDATE


Thursday, July 24, 2014

Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA


*** Monday, January 09, 2017 ***

*** Oral Transmission of L-Type Bovine Spongiform Encephalopathy Agent among Cattle ***

CDC Volume 23, Number 2—February 2017

Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.


ONE DECADE POST MAD COW FEED BAN OF AUGUST 1997...2007

10,000,000 POUNDS REASON Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

2007

Date: March 21, 2007 at 2:27 pm PST

RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II PRODUCT

Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007 CODE Cattle feed delivered between 01/12/2007 and 01/26/2007 RECALLING FIRM/MANUFACTURER Pfeiffer, Arno, Inc, Greenbush,
WI. by conversation on February 5, 2007.

Firm initiated recall is ongoing.

REASON Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE 42,090 lbs. DISTRIBUTION WI

___________________________________

PRODUCT Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007

CODE The firm does not utilize a code - only shipping documentation with commodity and weights identified.

RECALLING FIRM/MANUFACTURER Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007.

Firm initiated recall is complete.

REASON Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement.

VOLUME OF PRODUCT IN COMMERCE 9,997,976 lbs. DISTRIBUTION ID and NV

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007


Tuesday, December 23, 2014

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION


Sunday, December 15, 2013

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE


Tuesday, September 06, 2016

A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211 cattle following intracranial inoculation


Saturday, July 23, 2016

BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION SURVEILLANCE, TESTING, AND SRM REMOVAL UNITED STATE OF AMERICA UPDATE JULY 2016


Sunday, June 14, 2015 

Larry’s Custom Meats Inc. Recalls Beef Tongue Products That May Contain Specified Risk Materials BSE TSE Prion 

http://madcowusda.blogspot.com/2015/06/larrys-custom-meats-inc-recalls-beef.html

Tuesday, July 26, 2016

Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY 2016


Monday, June 20, 2016

Specified Risk Materials SRMs BSE TSE Prion Program


Wednesday, May 25, 2016

USDA APHIS National Scrapie TSE Prion Eradication Program April 2016 Monthly Report Prion 2016 Tokyo Update


Wednesday, December 21, 2016

TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 2016 ANNUAL REPORT ARS RESEARCH


Thursday, December 08, 2016

USDA APHIS National Scrapie Eradication Program October 2016 Monthly Report Fiscal Year 2017 atypical NOR-98 Scrapie



Tuesday, August 9, 2016

Concurrence with OIE Risk Designations for Bovine Spongiform Encephalopathy [Docket No. APHIS-2015-0055]

BILLING CODE: 3410-34-P DEPARTMENT OF AGRICULTURE Animal and Plant Health Inspection Service


MONDAY, JANUARY 4, 2016

Long live the OIE, or time to close the doors on a failed entity?


Long live the OIE, or time to close the doors on a failed entity?

IN A NUT SHELL ;

(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,


snip...see ;

Sunday, October 18, 2015

World Organisation for Animal Health (OIE) and the Institut Pasteur Cooperating on animal disease and zoonosis research


Thursday, December 17, 2015

Annual report of the Scientific Network on BSE-TSE 2015 EFSA-Q-2015-00738 10 December 2015



From: TSS (216-119-138-129.ipset18.wt.net)

Subject: FRANCE reveals new mad cow tally and it' NOT good...

Date: December 5, 2000 at 12:52 pm PST

Tuesday, 5 December, 2000, 11:32 GMT France reveals new mad cow tally

The BSE crisis is hitting more and more farmers France has revealed that four new cases of mad cow disease have been detected, taking the year's total to more than four times the 1999 figure.

Officials at the agriculture ministry said 125 cows suffering from the disease had now been found on farms across France since January.

Two new cases of the human form of the disease, vCJD, were also disclosed by health officials in the UK, taking the number to 87.

The news came a day after European agriculture ministers agreed tough new measures aimed at restoring public confidence in beef in Europe.

After a nine-hour meeting, the ministers banned all cattle over 30 months old from entering the food chain, unless individually tested and proved free of BSE.

That means the slaughter of thousands of cattle until adequate BSE tests are introduced next year.

The ministers also approved a six-month ban on all meat and bonemeal in animal feed.

Some BSE-free Scandinavian countries say that is over the top.

The Finnish delegation said the ban was unjustified on scientific and moral grounds.

But German Agriculture Minister Karl-Heinz Funke said the ban was too short.

Slaughtered

"It's not enough that the EU ban on meat-based feeds will initially last for six months," Mr Funke told WDR 2 radio.

Due to take effect in January, the ban does not include fishmeal used in pig and poultry feed, officials say.

The ban on cattle over 30 months old entering the food chain means that cattle can continue to be traded as usual, but once slaughtered the carcasses must be tested for BSE and cleared as fit for human consumption.

Failure will mean the carcasses being sent for incineration.

Panic

The cost of testing will be split between national authorities and Brussels, with the Commission picking up 70% of the bill.

The new scheme also involves extending the list of "specified risk materials" currently banned from human consumption - the brains, spinal cords and spleens of cattle - to include the whole intestine.

The ban, expected to cost $1.7bn, was approved by all 15 ministers.

The emergency Brussels meeting was in the wake of panic in France and Germany where BSE is on the increase.

National governments will get no EU help to pay for the recall and destruction of the meat and bonemeal in circulation.


November 21, 2000

A Mad Cow Is Just the Half of It

By DIANE JOHNSON

ARIS — The rage and dismay seizing France since tons of beef potentially infected with mad cow disease were found to have been sold in some supermarkets seems both warranted and excessive, like most public and political matters that involve national beliefs and betrayals of good faith.

What the French have learned is indeed alarming: that many thousands of tons of bone meal — the repulsive feed made of ground-up animals — from possibly infected English cattle were imported into France for more than two years after France embargoed British beef. It was fed to French chickens, pigs and fish, and often, to French cows. Three cases of the new variant of Creutzfeldt-Jakob's disease, the human form of mad cow disease, have been found in France; around 100 infected cows and a small percentage of seemingly healthy animals have tested positive.

France has several articles of faith that are being profoundly shaken, and it is this threat to comfortable assumptions, perhaps more than the disease itself, that is fueling the indignation in France.

One such assumption is that this sort of thing can be controlled. France, like many other European countries, thought that a ban on English beef would prevent the dreaded, if uncommon, disease from slipping into the country. This might have worked, were it not for mistakes and failings of various usual kinds — greed, lax enforcement, inability on the part of farmers to believe it could happen. To find out that the system was allowing an agent of transmission to be sold here and fed to French cattle — not to mention to other animals — has caused understandable panic, compounded by anger at officials and almost reflex French Anglophobia.

To date, a French person has a much greater chance of dying of smoking, that widely tolerated health threat, than of this exotic and rare disease. But things to do with nourishment have a central hold on any national psyche, and this is especially so in France, where food has a national identity and a focal place in the culture. The idea of French cuisine is based on sentimental ideas of French agriculture, fresh local produce, fine ingredients and so on.

The French also believe firmly that they are protected by a vigilant government. Certainly the government tends to protect the French state of mind. Maps of Europe after Chernobyl, depicting radiation levels in all adjacent countries, showed fallout miraculously stopping at the French border. In the current crisis, the French are shocked to find that French incompetence as much as English perfidy is to blame, and this has compounded the understandable fury with chagrin.

Since the discovery of the disease in France, indignation has swirled around all public officials. Beef has been withdrawn from the menus of schools and day care centers, while confusion about the science still reigns. For instance, many in France have not distinguished between "contaminated" and "genetically modified," foodstuffs, with one paper predicting darkly that the English, already feeding their animals with soybeans from America, are only substituting plague for cholera, implying the French should not make the same mistake.

Do we dare eat beef? the French are asking. People who have long followed French food production have remarked that this should serve as a wake-up call to a nation that is rapidly losing its tradition of excellent local products to factory farming and freeze-dried imports. Meantime, the Nouvel Observateur assessment about beef is: only from a "boucher serieux," a serious butcher who knows where the meat came from. But how many people have access to a serious butcher, even in France? At the very least it must now be clear to the French that in stopping the spread of this contaminant, pride and tradition do not suffice.

Diane Johnson is author, most recently, of "Le Mariage."


From: TSS (216-119-138-129.ipset18.wt.net)

Subject: Re: FRANCE reveals new mad cow tally and it' NOT good...

Date: December 5, 2000 at 12:58 pm PST

In Reply to: FRANCE reveals new mad cow tally and it' NOT good... posted by TSS on December 5, 2000 at 12:52 pm:

December 5, 2000

Europe Takes Toughest Steps to Fight Mad Cow Disease

By SUZANNE DALEY

ARIS, Dec. 4 — Moving to calm growing fears over mad cow disease, the European Union today voted its most drastic measures yet to try to control the spread of the fatal illness.

In a special emergency session, the union's agricultural ministers voted to ban the use of feed laced with animal products, not just for cattle but for all farm animals, for at least six months.

In addition, all cattle over the age of 30 months are to be removed from the food chain unless they can be tested to make sure they are disease free. As testing capacity is limited, this is likely to mean that two million head of cattle in the union's 15 member countries will be slaughtered.

Both measures are expected to be costly. Union officials estimate that the feed ban — intended to prevent cattle from eating, even accidentally, infected animal parts that can transmit the disease — will cost nearly $4 billion a year. The removal of older cattle from the food chain will cost another $800 million, officials said. Evidence of mad cow disease has never been found in young cattle.

"The crisis we have to come to grips with is an unusual one," said Franz Fischler, the European Union's agricultural minister, after emerging from the nine-hour meeting. "It needs unusual measures."

The measures come as most European countries have been struggling with a growing panic among consumers about the safety of beef. Wholesalers in several countries have reported a drop in sales of nearly 50 percent in the last few weeks.

The recent panic began in France, where the number of reported cases of mad cow has increased dramatically this year, in part due to increased testing. Last year, France counted 31 cases. This year, it already has more than 110.

But the panic soon spread throughout Europe — especially when Germany and Spain, which had never reported cases, said two weeks ago that they too had diseased cows.

Since then, health and agriculture officials have been scrambling to reassure consumers that everything is being done to protect them. Several countries have announced new measures of their own, including increased testing and new bans on beef from France. These bans are to be reviewed by the union's scientific steering committee to decide whether they are appropriate.

The European Union action tonight was devised to offer a Europe- wide approach to the problems. In many ways, it mirrors the actions that Britain took years ago to combat its own epidemic. Britain has recorded more than 170,000 cases of mad cow, far more than any other European country.

Much about mad cow disease, also known as bovine spongiform encephalopathy, remains a mystery. But scientists believe that cattle originally contracted the disease by eating feed made with tissue from sheep infected with a related neurological ailment, scrapie. Experts believe that the fatal disease it is caused by aberrant proteins called infectious prions, which leave the brain with spongelike holes.

More than 80 people have died in Britain and two in France from the fatal human equivalent, new variant Creutzfeldt-Jakob Disease, which has been linked to the infected beef. The incubation period is believed to be up to 25 years, and so health officials have warned that the toll may rise sharply.

European Union officials said tonight that the ban on animal parts in feed won easy approval from a majority of members, though some countries objected. Officials said Finland, for instance, did not want to apply the ban because it did not yet have any cases of the disease.

Much of the cost involved in banning the animal parts in the feed will be for storing and incinerating the three million tons of ground up bones and intestinal parts that are a natural waste product of Europe's meat industry and that have until now been used to enhance animal feed.

Some of the feed is used as fuel in cement factories, and there is some hope that more will be used for that purpose. But much of it may simply have to be destroyed.

Animal parts have been fed to cows and other farm animals as a source of protein since World War II. Banning them is likely to have have serious trade implications for the European Union as farmers will now have to find a way to replace it. One alternative would be to buy soy meal from the United States. But the ministers voted at the last minute to exclude fish meal from the ban, meaning farmers will still be able to feed those products to pigs and chickens, deflecting some of the potential need to import soy.


From: TSS (216-119-130-159.ipset10.wt.net)

Subject: FRANCE--BSE/CJD 'The Madness Spreads'

Date: November 10, 2000 at 2:11 pm PST

France To Ban Sweetbreads In Anti-Mad Cow Effort Ag Minister Criticizes Farm Union's Cattle Withdrawal Plan

PARIS, Nov. 10 (Associated Press) -- France plans to ban sweetbreads for a one-year period as a precautionary measure to fight the possible human consequences of mad cow disease, Agricultural Ministry officials said Friday.

The ban is to take effect shortly, said the ministry's special research mission for bovine spongiform encephalopathy, or mad cow disease.

The decision is based on advice from France's food safety agency, but does not mean that consumers who have recently eaten the popular product -- made from the cow's thymus gland -- should fear, said the mission.

It concerns cows born after May 1, 1999. Thymuses of cows born before that date were banned earlier. Cow intestines were banned last month.

The move came amid growing fears that mad cow disease could endanger France's beef-eaters with the human form of the brain-wasting ailment.

Scientists have linked made cow disease to a variant strand of Creutzfeldt-Jakob disease. Two people in France are known to have died from the malady -- compared to 81 people in Britain where the beef scare exploded in 1996.

Officials of France's leftist government have warned against what they call a psychosis among the French that has led to numerous towns banning beef from school cafeteria menus.

On Friday, Nantes University Hospital Center made known it was withdrawing beef from its maternity and pediatric units, RTL radio reported.

Agriculture Minister Jean Glavany criticized, in an interview published Friday, a plan by France's largest farmers' union to withdraw from the market cows born before July 1996. He said it has "no rational foundation" in the fight against mad cow disease and serves only to feed the psychosis.

"If it were a public health measure, I would absolutely support it," Glavany was quoted as saying in the daily Liberation. "But it's nothing of the sort."

The farmers' union known as the FNSEA said Tuesday it planned to withdraw from the market older cows, born before France imposed strict measures on cattle feed in July 1996 when mad cow disease became a palpable fear among Europeans because of its spread in Britain.

Glavany conceded that France's leftist coalition government had underestimated how a rising number of mad cow cases in France would effect public opinion.

More than 90 cases of mad cow disease have been detected in France this year, compared to 31 last year.

However, Glavany said the increase is due to special detection tests being carried out.

Beef prices have plunged at meat markets around France, and one slaughter house in Quimper, in the eastern Finistere region, which lost 65 percent of its business within a few days, may close its doors next week, France-Info radio reported.


French Beef Sales Plunge Amid Mad Cow Scare

PARIS, Nov. 10 (Xinhua News Agency) -- Sales of beef plunged by 42 percent on Thursday compared with the previous Thursdays in the food wholesale market of Rungi near Paris, which is Europe's largest, according to officials of cattle and food sale unions.

This is the second consecutive day for beef salers to suffer large losses, as the sales of beef fell by 41 percent on Wednesday amid a mad cow disease scare in the country and elsewhere in Europe.

The crisis mainly resulted from a halt by several European countries to buy French beef.

Poland, Hungary and Spain have suspended importing beef from France, while Russia has decreased its purchase.


Wednesday, December 14, 2016

Diagnosis of Human Prion Disease Using Real-Time Quaking-Induced Conversion Testing of Olfactory Mucosa and Cerebrospinal Fluid Samples


*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery ***

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC. Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr Bacliff, Tex

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.




Terry S. Singeltary Sr.

Monday, January 2, 2017

Bovine Spongiform Encephalopathy Induces Misfolding of Alleged Prion-Resistant Species Cellular Prion Protein without Altering Its Pathobiological Features


Articles, Neurobiology of Disease

Bovine Spongiform Encephalopathy Induces Misfolding of Alleged Prion-Resistant Species Cellular Prion Protein without Altering Its Pathobiological Features




Enric Vidal, Natalia Fernández-Borges, Belén Pintado, Montserrat Ordóñez, Mercedes Márquez, Dolors Fondevila, Juan María Torres, Martí Pumarola and Joaquín Castilla



Journal of Neuroscience 1 May 2013, 33 (18) 7778-7786; DOI: https://doi.org/10.1523/JNEUROSCI.0244-13.2013

Enric Vidal



3Centre de Recerca en Sanitat Animal, Campus de la Universitat Autònoma de Barcelona (UAB)-IRTA, 08193 Bellaterra, Barcelona, Spain,





Abstract




Bovine spongiform encephalopathy (BSE) prions were responsible for an unforeseen epizootic in cattle which had a vast social, economic, and public health impact. This was primarily because BSE prions were found to be transmissible to humans. Other species were also susceptible to BSE either by natural infection (e.g., felids, caprids) or in experimental settings (e.g., sheep, mice). However, certain species closely related to humans, such as canids and leporids, were apparently resistant to BSE. In vitro prion amplification techniques (saPMCA) were used to successfully misfold the cellular prion protein (PrPc) of these allegedly resistant species into a BSE-type prion protein. The biochemical and biological properties of the new prions generated in vitro after seeding rabbit and dog brain homogenates with classical BSE were studied. Pathobiological features of the resultant prion strains were determined after their inoculation into transgenic mice expressing bovine and human PrPC. Strain characteristics of the in vitro-adapted rabbit and dog BSE agent remained invariable with respect to the original cattle BSE prion, suggesting that the naturally low susceptibility of rabbits and dogs to prion infections should not alter their zoonotic potential if these animals became infected with BSE. This study provides a sound basis for risk assessment regarding prion diseases in purportedly resistant species.





snip...





Discussion




Prion diseases have been known for a long time, especially scrapie in sheep and goats, and some diseases affecting humans, including Kuru and Creutzfeldt-Jakob disease. Yet the occurrence and identification of the BSE epizootic was a remarkable milestone in the history of prion disease as cattle had never before been affected by TSE and so were presumed resistant. The recognition that BSE was zoonotic turned prion diseases of animals into a serious threat to public health, which resulted in an unprecedented crisis of confidence in consumers. Vast amounts of money were spent in numerous countries, not just to eliminate hundreds of thousands of infected animals, but also to establish radical changes related to disease control and surveillance, i.e., diagnostic tests, disposal of specified risk material, which could no longer be used in the food industry, etc. However, as prion diseases became the subject of profound scientific study, previously unknown prion strains have been discovered, many with unknown species susceptibilities and zoonotic potential. Thus, the susceptibility of different species to prions, particularly those that may come into close contact with humans, is a matter of continuous debate and study in the scientific community and great concern when determining health and safety policies.





Detailed study of naturally occurring prions is not enough to understand the behavior of these proteins in certain species. Before 1985, no one could have foreseen the massive cattle BSE epizootic. Hence, the scientific community is now particularly cautious when assessing the risk associated with host susceptibilities of various prions. Nevertheless, certain species display a limited or apparently null susceptibility to prion disease. Therefore, could one assume that such species would be resistant to all existing prion strains? If only one prion strain should be able to infect a new species, it might adapt to the new host and become easily transmissible. To address this we used all the tools available (i.e., in vitro amplification and transgenic mouse models) to evaluate the behavior of BSE prions in two historically considered prion disease resistant species: dogs and rabbits. BSE was chosen due to its zoonotic properties and because, as mentioned above, it is a promiscuous strain that has demonstrated infectivity in a wider range of species than most other prions. Even though these experimental conditions are far from modeling natural scenarios, they may enable us to predict the outcome of potential unforeseen species susceptibilities and epizootics as occurred with BSE (mad cow disease). During the last decade, in vitro replication studies endorsed PMCA as one of the most powerful technologies to overcome transmission barriers (Castilla et al., 2008; Green et al., 2008; Fernández-Borges and Castilla, 2010; Barria et al., 2011; Kurt et al., 2011; Yoshioka et al., 2011; Chianini et al., 2012). Thus, dog and rabbit normal brain homogenates were seeded with BSE prions and submitted to in vitro amplification (saPMCA) to try to overcome the species transmission barrier. Interestingly, rabbit PrPC could be misfolded with relative ease by any of the prion strains used TSE (Barlow and Rennie, 1976; Fernández-Borges et al., 2009; Chianini et al., 2012). This was rather surprising since this species had, until then, been considered resistant to TSE (Barlow and Rennie, 1976; Fernández-Borges et al., 2009; Chianini et al., 2012). However, dog brain homogenate could not be misfolded until the initial BSE prion seed concentration was raised considerably, and even with this modification only BSE was able to misfold dog PrPC, which suggested a strong resistance of this species to BSE.





Comparison of the original cattle BSE prions with those generated by seeding rabbit and dog brain homogenate with BSE followed by in vitro saPMCA demonstrated that the biochemical strain properties of BSE were maintained in the new species, i.e., glycoform proportion and molecular weight after PK digestion. This suggests that the BSE prion's conformation is reliably transmitted to new PrPC species in vitro. However, to ensure that the BSE strain's pathobiological properties was also transmitted, particularly those regarding infectivity and the ability to cross certain species barriers, an in vivo approach was mandatory. The transgenic mouse model expressing the bovine PRNP gene was chosen as it had previously demonstrated to reproduce reliably BSE strain features. This model was key in determining that BSE acquired a different behavior regarding the length of the incubation period upon its passage through sheep, while maintaining other strain features, such as Western blot electrophoretical profile, molecular weight, and PK resistance (Espinosa et al., 2007). As predicted by the in vitro experiments (once initially converted, BSE-DoPrPres and BSE-RaPrPres easily amplified further dog and rabbit normal brain homogenates in vitro), both rabbit and dog adapted BSE efficiently infected tgBov mice with attack rates of 100%. Initially the incubation period was slightly longer for both inocula than that of cattle BSE, probably due to a lack of adaptation of the in vitro generated prions to the new host (bovine) prion. However, on second passage the incubation periods were significantly shortened. Interestingly the behavior of BSE-DoPrPres was similar to that observed in sheep BSE where the biochemical, lesion, and immunohistochemical features were unchanged but with a reduction in the incubation period (Espinosa et al., 2007). The neuropathology and molecular signature of PrPres found in tgBov mice showed no significant differences between cattle BSE and the two in vitro-generated prions, suggesting that the strain features had not changed upon passage through rabbit and dog brain homogenates.





The data shown here prove that the difficulties found in misfolding certain species of PrPC molecules do not imply a loss of the pathobiological features encoded in the BSE structure. Compared with cat PrPC (data not shown), dog PrP was tremendously difficult to misfold in vitro. It could only be converted when a relatively large dose of BSE prion was used as seed. However, the similarity of dog PrP primary sequence, differing principally in just three residues (positions: 163, 181, and 189) with cat PrP (Lysek et al., 2005; Stewart et al., 2012), would predict a similar behavior to feline spongiform encephalopathy (FSE). In fact, FSE was one of the first nonbovine prions shown to maintain BSE strain features (Lezmi et al., 2006). Accordingly, once the bovine–canine barrier was surpassed, BSE pathobiological features remained stable in BSE-DoPrPres.





However, the results obtained with BSE cannot be used to predict the behavior of other prion strains when adapted to these species in vivo or in vitro. Thus, instead of a species transmission barrier a strain transmission barrier should be considered as suggested by Scott and coworkers (Scott et al., 2005). Prion strains might be classified according to their ability to transmit to different species and also in relation to their adaptability to new hosts and their permissiveness to change. Therefore, BSE would be a strain with a low (or null) permissiveness to change and yet high adaptability to different environments. As such, the zoonotic behavior of BSE toward humans can be predicted regardless of the host infected with the BSE prion. Any species derivation of BSE, be it rabbit-BSE, dog-BSE, or any other version, is likely to encode a structure capable of misfolding human PrPC. Second passage experiments in transgenic mice expressing human PrP (Tg340 mice) would have been useful to determine whether silent infection was present in mice inoculated with cattle BSE and BSE-DoPrPres which seems to be a plausible assumption according to experiments published in this model (Padilla et al., 2011).

Our in vitro and in vivo results predict that, hypothetically, if BSE infected canids and leporids (considered, so far, prion resistant) it would maintain its pathobiological features; including zoonotic potential. This is particularly relevant with respect to rabbit amplified BSE as rabbits are eaten by humans. In conclusion, it is strongly recommended that no mammalian species be fed with animal protein potentially contaminated with BSE to prevent a new epizootic and zoonosis of unknown consequences.





Given the difficulties of performing infectivity studies in many natural hosts (without previous in vitro replication), transgenic mice have been generated expressing rabbit and dog PRNP gene which have been inoculated with cattle BSE, among other strains of interest, to test the in vivo susceptibility of these PRNP sequences. In addition, a detailed study of the primary amino acid sequences of bovine PrP compared with rabbit and dog PrP, focusing on the structural peculiarities of each amino acid change, is ongoing to explain the different PrPC to PrPd conversion abilities of each species.



Footnotes




  • This work was financially supported by two national grants from Spain (AGL2009-11553-C02-01 and AGL2008-05296-C02), Basque Government Grant PI2010-18, and Etortek Research Programs 2011/2013. We thank the IKERBasque Foundation for their support. We thank CIC bioGUNE, Sierra Espinar, Marta Valle, Mariano Moreno, and Paola Marco for the providing the vivarium and maintenance; the CReSA Biocontainment Unit staff for care and maintenance of the animals; Tomás Mayoral for the bovine spongiform encephalopathy brain tissue samples; and Mark Daeglish for his critical revision of the paper.
  • The authors declare no competing financial interests.
  • Correspondence should be addressed to Dr. Joaquín Castilla, CIC bioGUNE, Parque tecnológico de Bizkaia, Derio 48160, Bizkaia, Spain. castilla@joaquincastilla.com










EP-021 Canine Prions: A New Form of Prion Disease



Mourad Tayebi1, Monique A David2, Brian Summers3



1 University of Melbourne, Veterinary Sciences, Australia; 2Ausbiologics, Sydney, Australia; 3Royal Veterinary College, London, UK



The origin of bovine spongiform encephalopathy (BSE), which rapidly evolved into a major epidemic remains unresolved and was initially widely attributed to transmission of sheep scrapie to cattle with contaminated feed prepared from rendered sheep carcasses. Alternative transmission hypotheses also include feed contaminated with unrecognized subclinical case(s) of bovine prion disease or with prion-infected human remains. However, following the demonstration of a BSE case exhibiting the novel mutation E211 K, similar to the E200K mutation associated with most genetic CJD in humans, support for a genetic origin of prion disease in cattle is gaining momentum. In contrast to other animal species such as feline, the canine species seems to be resistant to prion disease as no canine prion cases were previously reported.



We describe here three cases of Rottweiler puppy (called RWD cases) with neurological deficits and spongiform change. We used animal bioassays and in vitro studies to show efficient interspecies transmission of this novel canidae prion isolate to other species.



Biochemical studies revealed the presence of partially proteinase K (PK)-resistant fragment and immunohistochemistry displayed staining for PrPSc in the cerebral cortex. Importantly, interspecies transmission of canine PrPSc derived from RWD3 brain homogenates following inoculation of hamsters led to signs of prion disease and replication of PrPSc in brains, spinal cords and spleens of these animals.



These findings if confirmed by further cases of prion disease in canidae and regardless of the origin of the disease would have a major impact on animal and public health.



PRION 2016 TOKYO






*** DEFRA TO SINGELTARY ON HOUND STUDY AND BSE 2001 ***



DEFRA Department for Environment, Food & Rural Affairs



Area 307, London, SW1P 4PQ Telephone: 0207 904 6000 Direct line: 0207 904 6287 E-mail: h.mcdonagh.defra.gsi.gov.uk



GTN: FAX:



Mr T S Singeltary P.O. Box 42 Bacliff Texas USA 77518



21 November 2001



Dear Mr Singeltary



TSE IN HOUNDS



Thank you for e-mail regarding the hounds survey. I am sorry for the long delay in responding.



As you note, the hound survey remains unpublished. However the Spongiform Encephalopathy Advisory Committee (SEAC), the UK Government's independent Advisory Committee on all aspects related to BSE-like disease, gave the hound study detailed consideration at their meeting in January 1994. As a summary of this meeting published in the BSE inquiry noted, the Committee were clearly concerned about the work that had been carried out, concluding that there had clearly been problems with it, particularly the control on the histology, and that it was more or less inconclusive. However was agreed that there should be a re-evaluation of the pathological material in the study.



Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be gained from it. The Chairman concluded that there were varying opinions within the Committee on further work. It did not suggest any further transmission studies and thought that the lack of clinical data was a major weakness.



Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the authors felt that it would not stand up to r~eer review and hence it was never published. As noted above, and in the detailed minutes of the SEAC meeting in June 95, SEAC considered whether additional work should be performed to examine dogs for evidence of TSE infection. Although the Committee had mixed views about the merits of conducting further work, the Chairman noted that when the Southwood Committee made their recommendation to complete an assessment of possible spongiform disease in dogs, no TSEs had been identified in other species and hence dogs were perceived as a high risk population and worthy of study. However subsequent to the original recommendation, made in 1990, a number of other species had been identified with TSE ( e.g. cats) so a study in hounds was less



critical. For more details see- http://www.bseinquiry, gov.uk/files/yb/1995/06/21005001 .pdf



As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. Thus unfortunately, I am unable to help with your request to supply information on the hound survey directly. My only suggestion is that you contact one of the researchers originally involved in the project, such as Gerald Wells. He can be contacted at the following address.



Dr Gerald Wells, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey, KT 15 3NB, UK



You may also wish to be aware that since November 1994 all suspected cases of spongiform encephalopathy in animals and poultry were made notifiable. Hence since that date there has been a requirement for vets to report any suspect SE in dogs for further investigation. To date there has never been positive identification of a TSE in a dog.



I hope this is helpful



Yours sincerely 4



HUGH MCDONAGH BSE CORRESPONDENCE SECTION



======================================



HOUND SURVEY



I am sorry, but I really could have been a co-signatory of Gerald's minute.



I do NOT think that we can justify devoting any resources to this study, especially as larger and more important projects such as the pathogenesis study will be quite demanding.



If there is a POLITICAL need to continue with the examination of hound brains then it should be passed entirely to the VI Service.



J W WILESMITH Epidemiology Unit 18 October 1991



Mr. R Bradley



cc: Mr. G A H Wells






3.3. Mr R J Higgins in conjunction with Mr G A Wells and Mr A C Scott would by the end of the year, indentify the three brains that were from the ''POSITIVE'' end of the lesion spectrum.






TSE in dogs have not been documented simply because OF THE ONLY STUDY, those brain tissue samples were screwed up too. see my investigation of this here, and to follow, later follow up, a letter from defra, AND SEE SUSPICIOUS BRAIN TISSUE SAF's. ...TSS






TSE & HOUNDS



GAH WELLS (very important statement here...TSS)



HOUND STUDY



AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.



snip...






76 pages on hound study;



snip...






The spongiform changes were not pathognomonic (ie. conclusive proof) for prion disease, as they were atypical, being largely present in white matter rather than grey matter in the brain and spinal cord. However, Tony Scott, then head of electron microscopy work on TSEs, had no doubt that these SAFs were genuine and that these hounds therefore must have had a scrapie-like disease. I reviewed all the sections myself (original notes appended) and although the pathology was not typical, I could not exclude the possibility that this was a scrapie-like disorder, as white matter vacuolation is seen in TSEs and Wallerian degeneration was also present in the white matter of the hounds, another feature of scrapie.



38.I reviewed the literature on hound neuropathology, and discovered that micrographs and descriptive neuropathology from papers on 'hound ataxia' mirrored those in material from Robert Higgins' hound survey. Dr Tony Palmer (Cambridge) had done much of this work, and I obtained original sections from hound ataxia cases from him. This enabled me provisionally to conclude that Robert Higgins had in all probability detected hound ataxia, but also that hound ataxia itself was possibly a TSE. Gerald Wells confirmed in 'blind' examination of single restricted microscopic fields that there was no distinction between the white matter vacuolation present in BSE and scrapie cases, and that occurring in hound ataxia and the hound survey cases.



39.Hound ataxia had reportedly been occurring since the 1930's, and a known risk factor for its development was the feeding to hounds of downer cows, and particularly bovine offal. Circumstantial evidence suggests that bovine offal may also be causal in FSE, and TME in mink. Despite the inconclusive nature of the neuropathology, it was clearly evident that this putative canine spongiform encephalopathy merited further investigation.



40.The inconclusive results in hounds were never confirmed, nor was the link with hound ataxia pursued. I telephoned Robert Higgins six years after he first sent the slides to CVL. I was informed that despite his submitting a yearly report to the CVO including the suggestion that the hound work be continued, no further work had been done since 1991. This was surprising, to say the very least.



41.The hound work could have provided valuable evidence that a scrapie-like agent may have been present in cattle offal long before the BSE epidemic was recognised. The MAFF hound survey remains unpublished.



Histopathological support to various other published MAFF experiments



42.These included neuropathological examination of material from experiments studying the attempted transmission of BSE to chickens and pigs (CVL 1991) and to mice (RVC 1994).






It was thought likely that at least some, and probably all, of the cases in zoo animals were caused by the BSE agent. Strong support for this hypothesis came from the findings of Bruce and others (1994) ( Bruce, M.E., Chree, A., McConnell, I., Foster, J., Pearson, G. & Fraser, H. (1994) Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and species barrier. Philosophical Transactions of the Royal Society B 343, 405-411: J/PTRSL/343/405 ), who demonstrated that the pattern of variation in incubation period and lesion profile in six strains of mice inoculated with brain homogenates from an affected kudu and the nyala, was similar to that seen when this panel of mouse strains was inoculated with brain from cattle with BSE. The affected zoo bovids were all from herds that were exposed to feeds that were likely to have contained contaminated ruminant-derived protein and the zoo felids had been exposed, if only occasionally in some cases, to tissues from cattle unfit for human consumption.



snip...








NEW URL ;








Friday, March 8, 2013



Dogs may have been used to make Petfood and animal feed






Monday, March 26, 2012



CANINE SPONGIFORM ENCEPHALOPATHY: A NEW FORM OF ANIMAL PRION DISEASE






Monday, February 14, 2011



THE ROLE OF PREDATION IN DISEASE CONTROL: A COMPARISON OF SELECTIVE AND NONSELECTIVE REMOVAL ON PRION DISEASE DYNAMICS IN DEER



NO, NO, NOT NO, BUT HELL NO !



Journal of Wildlife Diseases, 47(1), 2011, pp. 78-93 © Wildlife Disease Association 2011








Monday, March 8, 2010



Canine Spongiform Encephalopathy aka MAD DOG DISEASE








snip...see full text ;





Friday, May 27, 2016



Canine Prions: A New Form of Prion Disease EP-021 PRION 2016 TOKYO








Monday, August 8, 2011




Susceptibility of Domestic Cats to CWD Infection








Monday, August 8, 2011




Susceptibility of Domestic Cats to CWD Infection




Oral.29: Susceptibility of Domestic Cats to CWD Infection

Amy Nalls, Nicholas J. Haley, Jeanette Hayes-Klug, Kelly Anderson, Davis M. Seelig, Dan S. Bucy, Susan L. Kraft, Edward A. Hoover and Candace K. Mathiason†

Colorado State University; Fort Collins, CO USA†Presenting author; Email: ckm@lamar.colostate.edu

Domestic and non-domestic cats have been shown to be susceptible to one prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted through consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free ranging felids scavenge cervid carcasses, including those in CWD affected areas, we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between 40–43 months following IC inoculation, two cats developed mild but progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on the brain of one of these animals (vs. two age-matched controls) performed just before euthanasia revealed increased ventricular system volume, more prominent sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere and in cortical grey distributed through the brain, likely representing inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles were demonstrated in the brains of both animals by immunodetection assays. No clinical signs of TSE have been detected in the remaining primary passage cats after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5) of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC inoculated cats are demonstrating abnormal behavior including increasing aggressiveness, pacing, and hyper responsiveness. Two of these cats have developed rear limb ataxia. Although the limited data from this ongoing study must be considered preliminary, they raise the potential for cervid-to-feline transmission in nature.

http://www.prion2011.ca/files/PRION_2011_-_Posters_(May_5-11).pdf


Hunting and diet


A successful generalist predator, the cougar will eat any animal it can catch, from insects to large ungulates (over 500 kg). Like all cats, it is an obligate carnivore, feeding only on meat. The mean weight of vertebrate prey (MWVP) was positively correlated (r=0.875) with puma body weight and inversely correlated (r=-0.836) with food niche breadth in all America. In general, MWVP was lower in areas closer to the Equator.[3] Its most important prey species are various deer species, particularly in North America; mule deer, white-tailed deer, elk, and even large moose are taken by the cat. Other species such as Bighorn Sheep, wild horses of Arizona, domestic horses, and domestic livestock such as cattle and sheep are also primary food bases in many areas.[38] A survey of North America research found 68% of prey items were ungulates, especially deer. Only the Florida Panther showed variation, often preferring feral hogs and armadillos.[3]

Shown eating. Cougars are ambush predators, feeding mostly on deer and other mammals. Investigation in Yellowstone National Park showed that elk, followed by mule deer, were the cougar's primary targets; the prey base is shared with the park's gray wolves, with whom the cougar competes for resources.[39] Another study on winter kills (November–April) in Alberta showed that ungulates accounted for greater than 99% of the cougar diet. Learned, individual prey recognition was observed, as some cougars rarely killed bighorn sheep, while others relied heavily on the species.[40]

http://en.wikipedia.org/wiki/Cougar


Oral.22:

Transmission and Pathogenesis of Chronic Wasting Disease in Cervid and Non-Cervid Species

Edward Hoover,† Candace K. Mathiason, Nicholas J. Haley, Timothy D. Kurt, Davis M. Seelig, Amy V. Nalls, Mark D. Zabel, Glenn C. Telling Department of Microbiology, Immunology, and Pathology; Colorado State University; Fort Collins, CO; Department of Microbiology, Immunology and Molecular Genetics and Neurology; University of Kentucky Medical Center; Lexington, KY USA †Presenting author

Now recognized in 18 states in the US, two Canadian provinces, and one Asian country, efficient horizontal transmission is a signature trait of chronic wasting disease (CWD) of cervids. The facile spread of CWD appears linked to the prion/host relationship facilitating efficient mucosal uptake, peripheral lymphoreticular amplification, and horizontal dissemination exploiting excretory tissues and their products. In addition, recent studies suggest the likelihood of early life mother to offspring transmission. Growing evidence from studies of cervid CWD exposure by natural routes indicate that the incubation period for overt infection detection and disease onset (if any) may be much longer than originally thought. Whether non-cervid species (including humans) may be susceptible to CWD infection and/or act as reservoirs for infection in nature remains unknown. In vitro and in vivo studies of the CWD species barrier indicate the potential for a host range extending beyond cervid species, although no evidence for this has thus far been detected in nature. Interestingly, rodent and mustelid species sympatric with free ranging cervids have been shown susceptible to CWD prions and such trans-species infection broadens the host range/strain characteristics of CWD prions. While the origins of CWD remain unknown, the relationship between sheep scrapie and CWD and the existence of multiple CWD prion strains/quasispecies remain interesting and merit further investigation.

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Monday, August 8, 2011

Susceptibility of Domestic Cats to CWD Infection

http://felinespongiformencephalopathyfse.blogspot.com/2011/08/susceptibility-of-domestic-cats-to-cwd.html



UPDATED DATA ON 2ND CWD STRAIN

Wednesday, September 08, 2010

CWD PRION CONGRESS SEPTEMBER 8-11 2010

http://chronic-wasting-disease.blogspot.com/2010/09/cwd-prion-2010.html




Sunday, November 01, 2009

American crows (Corvus brachyrhynchos) and potential spreading of CWD through feces of digested infectious carcases

http://chronic-wasting-disease.blogspot.com/2009/11/american-crows-corvus-brachyrhynchos.html




Monday, July 13, 2009

Deer Carcass Decomposition and Potential Scavenger Exposure to Chronic Wasting Disease

http://chronic-wasting-disease.blogspot.com/2009/07/deer-carcass-decomposition-and.html



 


Wednesday, December 21, 2016


 


TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 2016 ANNUAL REPORT ARS RESEARCH


 


http://transmissiblespongiformencephalopathy.blogspot.com/2016/12/transmission-differentiation-and.html


 


http://transmissiblespongiformencephalopathy.blogspot.com/


 


 


Thursday, December 08, 2016


 


USDA APHIS National Scrapie Eradication Program October 2016 Monthly Report Fiscal Year 2017 atypical NOR-98 Scrapie


 


http://scrapie-usa.blogspot.com/2016/12/usda-aphis-national-scrapie-eradication.html


 


http://scrapie-usa.blogspot.com/


 


 


Chronic Wasting Disease CWD TSE Prion Cervid


 


http://chronic-wasting-disease.blogspot.com/


 


 


Creutzfeldt Jakob Disease CJD


 


http://creutzfeldt-jakob-disease.blogspot.com/  


 


 


kind regards, terry