From: Terry S. Singeltary Sr.
Sent: Monday, May 05, 2014 11:54 AM
To: BSE-L BSE-L
Cc: CJD-L ; CJDVOICE CJDVOICE ; bloodcjd bloodcjd
Subject: Brazil BSE Mad Cow disease confirmed OIE 02/05/2014
Bovine spongiform encephalopathy, Brazil
Information received on 02/05/2014 from Dr Figueiredo Marques Guilherme
Henrique , Director, Departamento de Saúde Animal , Ministério da Agricultura,
Pecuaria e Abastecimento , Brasilia, Brazil
Summary
Report type Immediate notification
Date of start of the event 19/03/2014
Date of pre-confirmation of the event 14/04/2014
Report date 02/05/2014
Date submitted to OIE 02/05/2014
Reason for notification Reoccurrence of a listed disease
Date of previous occurrence 19/12/2010
Manifestation of disease Sub-clinical infection
Causal agent Prion
Nature of diagnosis Laboratory (advanced)
This event pertains to the whole country
New outbreaks (1)
Outbreak 1 Porto Esperidião, MATO GROSSO
Date of start of the outbreak 19/03/2014
Outbreak status Resolved (01/05/2014)
Epidemiological unit Farm
Affected animals
Species Susceptible Cases Deaths Destroyed Slaughtered
Cattle 1177 1 0 50 0
Buffaloes 11 0 0 0 0
Affected population A 12-year-old female bovine tested during emergency
slaughter at the slaughterhouse. The animal was born and raised on a full-cycle
beef farm on extensive grazing, with a population of 1,177 cattle and 11
buffaloes.
Summary of outbreaks Total outbreaks: 1
Total animals affected
Species Susceptible Cases Deaths Destroyed Slaughtered
Cattle 1177 1 0 50 0
Buffaloes 11 0 0 0 0
Outbreak statistics
Species Apparent morbidity rate Apparent mortality rate Apparent case
fatality rate Proportion susceptible animals lost*
Cattle 0.08% 0.00% 0.00% 4.25%
Buffaloes 0.00% 0.00% - 0.00%
*Removed from the susceptible population through death, destruction and/or
slaughter
Epidemiology
Source of the outbreak(s) or origin of infection •Unknown or inconclusive
Epidemiological comments As part of the Brazilian surveillance system for
Bovine Spongiform Encephalopathy (BSE), the prion marker was identified on 14
April 2014 in a 12-year-old female bovine sent for emergency slaughter because
she was found fallen at her arrival at the slaughterhouse following some
problems during transport. The animal was born and raised in the same full-cycle
beef farm on extensive grazing. Meat and other products from this animal did not
enter the food chain and there was no risk for human population. Tracing back
animal movements since 2000, it was established that some animals from the birth
cohort of this animal had been moved to 10 other properties in 3 municipalities
in the state of Mato Grosso. During the epidemiological investigation, 49
animals from the cohort, which did not show clinical signs of the disease, were
destroyed. Samples of nervous tissue were taken from the cohort animals and
tested for BSE at the National Laboratory and all were negative on 1 May 2014.
All control measures according to the OIE Terrestrial Animal Health Code have
already been applied in order to close the outbreak and only the results of the
typing tests carried out at the Reference Laboratory at Weybridge (United
Kingdom) are pending.
Control measures
Measures applied •Quarantine •Screening •Modified stamping out •No
vaccination •No treatment of affected animals
Measures to be applied •No other measures
Diagnostic test results
Laboratory name and type Species Test Test date Result
National Agricultural Laboratory (LANAGRO/PE) (National laboratory) Cattle
immunohistochemical test 14/04/2014 Positive
National Agricultural Laboratory (LANAGRO/PE) (National laboratory) Cattle
immunohistochemical test 01/05/2014 Negative
Animal Health and Veterinary Laboratories Agency (AHVLA), Weybridge, United
Kingdom (OIE’s Reference Laboratory) Cattle immunohistochemical test 01/05/2014
Positive
Future Reporting
The event is continuing. Weekly follow-up reports will be submitted.
Map of outbreak locations
>>>Atypical BSE, or “mad cow” disease, is a form of
the prion disease not associated with the animal’s consumption of
feed.<<<
don't you just love it when the officials just make stuff up $$$
*** What irks many scientists is the USDA’s April 25 statement that the
rare disease is “not generally associated with an animal consuming infected
feed.”
The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul
Brown, one of the world’s experts on this type of disease who retired recently
from the National Institutes of Health. "(The agency) has no foundation on which
to base that statement.”
The present study demonstrated successful intraspecies transmission of
H-type BSE to cattle and the distribution and immunolabeling patterns of PrPSc
in the brain of the H-type BSE-challenged cattle. TSE agent virulence can be
minimally defined by oral transmission of different TSE agents (C-type, L-type,
and H-type BSE agents) [59]. Oral transmission studies with H-type BSE infected
cattle have been initiated and are underway to provide information regarding the
extent of similarity in the immunohistochemical and molecular features before
and after transmission.
In addition, the present data will support risk assessments in some
peripheral tissues derived from cattle affected with H-type BSE.
*** 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. ***
P.9.21
Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim
McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre,
Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of
Calgary, Canada
Background: Three BSE types (classical and two atypical) have been
identified on the basis of molecular characteristics of the misfolded protein
associated with the disease. To date, each of these three types have been
detected in Canadian cattle. Objectives: This study was conducted to further
characterize the 16 Canadian BSE cases based on the biochemical properties of
there associated PrPres.
Methods: Immuno-reactivity, molecular weight, glycoform profiles and
relative proteinase K sensitivity of the PrPres from each of the 16 confirmed
Canadian BSE cases was determined using modified Western blot analysis.
Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type
and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and
changes in glycosylation similar to other atypical BSE cases. PK digestion under
mild and stringent conditions revealed a reduced protease resistance of the
atypical cases compared to the C-type cases. N terminal- specific antibodies
bound to PrPres from H type but not from C or L type. The C-terminal-specific
antibodies resulted in a shift in the glycoform profile and detected a fourth
band in the Canadian H-type BSE.
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
Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of
BSE in Canada Singeltary reply ;
P.4.23
Transmission of atypical BSE in humanized mouse models
Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3,
Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5,
Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto
Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany;
4National Veterinary Research Institute, Poland; 5Kansas State University
(Previously at USDA National Animal Disease Center), USA
Background: Classical BSE is a world-wide prion disease in cattle, and the
classical BSE strain (BSE-C) has led to over 200 cases of clinical human
infection (variant CJD). Atypical BSE cases have been discovered in three
continents since 2004; they include the L-type (also named BASE), the H-type,
and the first reported case of naturally occurring BSE with mutated bovine PRNP
(termed BSE-M). The public health risks posed by atypical BSE were largely
undefined.
Objectives: To investigate these atypical BSE types in terms of their
transmissibility and phenotypes in humanized mice. Methods: Transgenic mice
expressing human PrP were inoculated with several classical (C-type) and
atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation
time, characteristics and distribution of PrPSc, symptoms, and histopathology
were or will be examined and compared.
Results: Sixty percent of BASE-inoculated humanized mice became infected
with minimal spongiosis and an average incubation time of 20-22 months, whereas
only one of the C-type BSE-inoculated mice developed prion disease after more
than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse
brains was biochemically different from bovine BASE or sCJD. PrPSc was also
detected in the spleen of 22% of BASE-infected humanized mice, but not in those
infected with sCJD. Secondary transmission of BASE in the humanized mice led to
a small reduction in incubation time.*** The atypical BSE-H strain is also
transmissible with distinct phenotypes in the humanized mice, but no BSE-M
transmission has been observed so far.
Discussion: Our results demonstrate that BASE is more virulent than
classical BSE, has a lymphotropic phenotype, and displays a modest transmission
barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg
mice. The possibility of more than two atypical BSE strains will be discussed.
Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.
P26 TRANSMISSION OF ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN
HUMANIZED MOUSE MODELS
Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina
Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi
Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case
Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto
Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany;
4National Veterinary Research Institute, Poland; 5Kansas State University,
Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous
address: USDA National Animal Disease Center, Ames, IA 50010, USA
Classical BSE is a world-wide prion disease in cattle, and the classical
BSE strain (BSE-C) has led to over 200 cases of clinical human infection
(variant CJD). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have
been discovered in three continents since 2004. The first case of naturally
occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006
in the USA. The transmissibility and phenotypes of these atypical BSE
strains/isolates in humans were unknown. We have inoculated humanized transgenic
mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M
isolate. We have found that the atypical BSE-L strain is much more virulent than
the classical BSE-C. *** The atypical BSE-H strain is also transmissible in the
humanized transgenic mice with distinct phenotype, but no transmission has been
observed for the BSE-M isolate so far.
III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE,
DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)
UPDATE
I ask Professor Kong ; Thursday, December 04, 2008 3:37 PM
Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform
Encephalopathies (BSE): Public Health Risk Assessment
''IS the h-BSE more virulent than typical BSE as well, or the same as
cBSE, or less virulent than cBSE? just curious.....''
Professor Kong reply ;
.....snip
''As to the H-BSE, we do not have sufficient data to say one way or
another, but we have found that H-BSE can infect humans. I hope we could publish
these data once the study is complete. Thanks for your interest.''
Best regards, Qingzhong Kong, PhD Associate Professor Department of
Pathology Case Western Reserve University Cleveland, OH 44106 USA END...TSS
Thursday, December 04, 2008 2:37 PM
"we have found that H-BSE can infect humans."
personal communication with Professor Kong. ...TSS
BSE-H is also transmissible in our humanized Tg mice. The possibility of
more than two atypical BSE strains will be discussed.
Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.
please see below from PRION2013 ;
*** This study imply the possibility that the novel BSE prions with high
virulence in cattle will be emerged during intraspecies transmission.
AD.56: The emergence of novel BSE prions by serial passages of H-type BSE
in bovinized mice
Kentaro Masujin, Naoko Tabeta, Ritsuko Miwa, Kohtaro Miyazawa, Hiroyuki
Okada, Shirou Mohri and Takashi Yokoyama National Institute of Animal Health;
Tsukuba, Japan
H-type bovine spongiform encephalopathy (BSE) is an atypical form of BSE,
and has been detected in several European countries, and North America.
Transmission studies of H-type BSE led to the emergence of the classical BSE
(C-BSE) phenotypes during passages in inbred wild type and bovinized
PrP-overexpressing transgenic mice. In this study, we conducted serial passages
of Canadian H-type BSE isolate in bovinized PrP-overexpressing transgenic mice
(TgBoPrP). H-type BSE isolate was transmitted to TgBoPrP with incubation periods
of 320 ± 12.2 d at primary passage. The incubation period of 2nd and 3rd passage
were constant (~= 220 d), no clear differences were observed in their biological
and biochemical properties. However, at the forth passage, 2 different BSE
phenotypes were confirmed; one is shorter survival times (109 ± 4 d) and the
other is longer survival times. TgBoPrP mice with longer incubation period
showed the H-type phenotype of PrPsc profile and pathology. However, those of
shorter incubation period were different phenotypes from previously existed BSE
prions (C-BSE, L-type BSE, and H-type BSE).
*** This study imply the possibility that the novel BSE prions with high
virulence in cattle will be emerged during intraspecies transmission.
www.landesbioscience.com
please see ;
Thursday, August 15, 2013
The emergence of novel BSE prions by serial passages of H-type BSE in
bovinized mice
LET'S take a closer look at this new prionpathy or prionopathy, and then
let's look at the g-h-BSEalabama mad cow. This new prionopathy in humans? the
genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_
mad cow in the world to date like this, ......wait, it get's better. this new
prionpathy is killing young and old humans, with LONG DURATION from onset of
symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and
the plaques are very similar in some cases too, bbbut, it's not related to the
g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that
they claim is a genetic TSE, has no relation to any gene mutation in that
family. daaa, ya think it could be related to that mad cow with the same genetic
make-up ??? there were literally tons and tons of banned mad cow protein in
Alabama in commerce, and none of it transmitted to cows, and the cows to humans
there from ??? r i g h t $$$ ALABAMA MAD COW g-h-BSEalabama In this study, we
identified a novel mutation in the bovine prion protein gene (Prnp), called
E211K, of a confirmed BSE positive cow from Alabama, United States of America.
This mutation is identical to the E200K pathogenic mutation found in humans with
a genetic form of CJD. This finding represents the first report of a confirmed
case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We
hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in
"the approximately 10-year-old cow" carrying the E221K mutation.
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and
VPSPr PRIONPATHY (see mad cow feed in COMMERCE IN ALABAMA...TSS)
her healthy calf also carried the mutation (J. A. Richt and S. M. Hall
PLoS Pathog. 4, e1000156; 2008).
This raises the possibility that the disease could occasionally be genetic
in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the
UK epidemic had most likely originated from such a mutation and argued against
the scrapierelated assumption. Such rare potential pathogenic PRNP mutations
could occur in countries at present considered to be free of BSE, such as
Australia and New Zealand. So it is important to maintain strict surveillance
for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many
countries still feed ruminant proteins to pigs). Removal of specified risk
material, such as brain and spinal cord, from cattle at slaughter prevents
infected material from entering the human food chain. Routine genetic screening
of cattle for PRNP mutations, which is now available, could provide additional
data on the risk to the public. Because the point mutation identified in the
Alabama animals is identical to that responsible for the commonest type of
familial (genetic) CJD in humans, it is possible that the resulting infective
prion protein might cross the bovine-human species barrier more easily. Patients
with vCJD continue to be identified. The fact that this is happening less often
should not lead to relaxation of the controls necessary to prevent future
outbreaks. Malcolm A. Ferguson-Smith Cambridge University Department of
Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail:
maf12@cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State
University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA NATURE|Vol
457|26 February 2009
P.9.21 Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim
McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre,
Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of
Calgary, Canada
Background: Three BSE types (classical and two atypical) have been
identified on the basis of molecular characteristics of the misfolded protein
associated with the disease. To date, each of these three types have been
detected in Canadian cattle.
Objectives: This study was conducted to further characterize the 16
Canadian BSE cases based on the biochemical properties of there associated
PrPres.
Methods: Immuno-reactivity, molecular weight, glycoform profiles and
relative proteinase K sensitivity of the PrPres from each of the 16 confirmed
Canadian BSE cases was determined using modified Western blot analysis. Results:
Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L
type. The Canadian H and L-type BSE cases exhibited size shifts and changes in
glycosylation similar to other atypical BSE cases. PK digestion under mild and
stringent conditions revealed a reduced protease resistance of the atypical
cases compared to the C-type cases. N terminal- specific antibodies bound to
PrPres from H type but not from C or L type. The C-terminal-specific antibodies
resulted in a shift in the glycoform profile and detected a fourth band in the
Canadian H-type BSE.
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.
Saturday, August 14, 2010
***BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama)
and VPSPr PRIONPATHY
*** (see mad cow feed in COMMERCE IN ALABAMA...TSS)
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
kind regards, terry
UPDATE SEE R-CALF CONCERNS
Brazil's Second Reported BSE Case Raises Food Safety Concerns
Billings, Mont. - On Monday the World Organization for Animal Health (OIE)
provided notice that Brazil confirmed its second case of bovine spongiform
encephalopathy (BSE), this time in a 12-year-old Brazilian cow. While the notice
states that none of the meat or other products from the infected cow entered the
food chain, a recent audit report by the U.S. Department of Agriculture (USDA)
reveals that Brazil has not been complying with BSE safeguard measures required
by the United States.
A recent audit report
by the USDA Food Safety and Inspection Service (FSIS) sent to the
Brazilian government on April 16, 2014, reveals that Brazil has not been
consistently implementing the United States' mandatory requirement that all
specified risk materials (SRMs) from cattle be excluded from the human food
chain as a condition for allowing Brazil to export beef to the United States.
Specifically, the audit found that beginning in early 2007, the Brazilian
government relaxed its SRM removal policies by issuing a notice that removed the
skull, trigeminal ganglia, vertebral column, and dorsal root ganglia in cattle
30 months of age or older from the list of SRMs that must be removed at
slaughter. The tissues improperly removed from the list of SRMs by the Brazilian
government are tissues known to harbor the BSE agent in infected cattle.
United States food safety inspectors confirmed that Brazil was not
routinely removing all high-risk tissues as required for countries that export
to the United States.
Despite Brazil's failure to meet U.S. food safety standards, FSIS
officials nevertheless determined that Brazil "continues to meet FSIS
equivalence criteria at an adequate level for this component (the SRM removal
component)."
R-CALF USA CEO Bill Bullard said these facts demonstrate the need to fully
enforce the U.S. country-of-origin labeling (COOL) law. "Only with COOL can
consumers choose to avoid purchasing their food from countries with questionable
food safety systems," he said.
Bullard also said these facts along with USDA's current plan to begin
importing beef from Brazilian states that are not free of foot-and-mouth disease
(FMD) is deeply troubling.
"More and more the USDA is demonstrating its unwillingness to prioritize
food safety and animal health above its politically motivated trade relations
goals," he concluded.
# # #
R-CALF USA (Ranchers-Cattlemen Action Legal Fund, United Stockgrowers of
America) is the largest producer-only cattle trade association in the United
States. It is a national, nonprofit organization dedicated to ensuring the
continued profitability and viability of the U.S. cattle industry. For more
information, visit www.r-calfusa.com or, call 406-252-2516.
Thursday, April 24, 2014
Brazil investigates possible BSE mad cow case
Thursday, September 26, 2013
Brazil evaluate the implementation of health rules on animal by-products
and derived products SRM BST TSE PRION aka MAD COW DISEASE
Friday, December 07, 2012
ATYPICAL BSE BRAZIL 2010 FINALLY CONFIRMED OIE 2012
Wednesday, December 19, 2012
Scientific Report of the European Food Safety Authority on the Assessment
of the Geographical BSE Risk (GBR) of Brazil
TSS
