Infected sheep may have come from U.S., not Ontario farm where officials 
slaughtered flock, court hears 
 
Adrian Humphreys | February 27, 2015 | Last Updated: Mar 1 5:07 PM ET 
 
The bizarre case of a flock of rare sheep — purportedly stolen from an 
Ontario farm by agricultural activists to thwart a federal kill order during a 
disease scare — was adjourned after government documents suggested the infected 
sheep that sparked the high-profile standoff could have actually been an animal 
from the United States.
 
Internal documents from the Canadian Food Inspection Agency (CFIA) also 
suggest workers may have tried to cover up any potential mistake or withheld 
information from its own reports, defence lawyers complain.
 
However, until more of the government’s records on the controversial case 
are released, it is difficult to know precisely what has gone on since 2010, 
when a sheep tested positive for scrapie, a degenerative disease in sheep 
similar to the “mad cow disease” that affects cattle.
 
Whether the diseased sheep came from the Ontario ewe, as the CFIA publicly 
says, or an American ram, as documents in court suggest it was once thought, is 
important not only for the criminal case but also for cross-border agricultural 
trade. 
 
On Monday, a 22-page letter from Shawn Buckley, a B.C. lawyer defending 
sheep owner Montana Jones against criminal charges, was submitted in court 
asking for an adjournment until the government can provide fuller 
documentation.
 
An internal CFIA email is quoted in Mr. Buckley’s letter saying: “The 
tattoo on the animal indicates it was imported from the USA — may be interesting 
… since this [is] a male and imported the focus goes to its herd of origin and 
therefore doesn’t require much on this farm in Canada.”
 
The sheep on Ms. Jones’ farm was a female and had never been to the U.S., 
Ms. Jones said. 
 
snip...
 
“We’ve got major concerns about disclosure in this case,” Mr. Buckley said 
in an interview. “A key factor is going to be them being able to try to prove 
that a sheep from Montana’s farm came down with scrapie in Alberta. And with the 
disclosure I have to date there are holes — there are huge holes.”
 
Judge Lorne Chester ordered the adjournment until April 27 to allow time 
for the government to provide more of its internal documents.
 
The sheep case was strange from the start.
 
In 2010, a sheep in Alberta tested positive for scrapie and the CFIA 
started an investigation. The CFIA then declared the sheep came from Ms. Jones’ 
farm in Hastings, 170 kilometres east of Toronto, where she bred Shropshire 
Sheep, a rare breed that traces its lineage back to the first sheep imported to 
Canada from England.
 
The CFIA moved to slaughter her flock. She fought to save them, often 
through emotional standoffs.
 
Before CFIA officers and police arrived at her farm in 2012 with an order 
to destroy 31 sheep, including 20 pregnant ewes, the flock went missing during 
the night. 
 
 
Tuesday, December 16, 2014 
 
Evidence for zoonotic potential of ovine scrapie prions 
 
Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves 
Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle 
Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia 
Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, 
Affiliations Contributions Corresponding author Journal name: Nature 
Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 
Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 
Article tools Citation Reprints Rights & permissions Article metrics 
 
Abstract 
 
Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant 
Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie 
prions remains unknown. Mice genetically engineered to overexpress the human 
prion protein (tgHu) have emerged as highly relevant models for gauging the 
capacity of prions to transmit to humans. These models can propagate human 
prions without any apparent transmission barrier and have been used used to 
confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie 
prions transmit to several tgHu mice models with an efficiency comparable to 
that of cattle BSE. The serial transmission of different scrapie isolates in 
these mice led to the propagation of prions that are phenotypically identical to 
those causing sporadic CJD (sCJD) in humans. These results demonstrate that 
scrapie prions have a zoonotic potential and raise new questions about the 
possible link between animal and human prions.
 
Subject terms: Biological sciences• Medical research At a glance
 
 
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 
 
 
Suspect symptoms
 
What if you can catch old-fashioned CJD by eating meat from a sheep 
infected with scrapie?
 
28 Mar 01 Most doctors believe that sCJD is caused by a prion protein 
deforming by chance into a killer. But Singeltary thinks otherwise. He is one of 
a number of campaigners who say that some sCJD, like the variant CJD related to 
BSE, is caused by eating meat from infected animals. Their suspicions have 
focused on sheep carrying scrapie, a BSE-like disease that is widespread in 
flocks across Europe and North America.
 
Now scientists in France have stumbled across new evidence that adds weight 
to the campaigners' fears. To their complete surprise, the researchers found 
that one strain of scrapie causes the same brain damage in mice as sCJD.
 
"This means we cannot rule out that at least some sCJD may be caused by 
some strains of scrapie," says team member Jean-Philippe Deslys of the French 
Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, 
south-west of Paris. Hans Kretschmar of the University of Göttingen, who 
coordinates CJD surveillance in Germany, is so concerned by the findings that he 
now wants to trawl back through past sCJD cases to see if any might have been 
caused by eating infected mutton or lamb...
 
2001
 
Suspect symptoms 
 
What if you can catch old-fashioned CJD by eating meat from a sheep 
infected with scrapie? 
 
28 Mar 01 
 
Like lambs to the slaughter 
 
31 March 2001 
 
by Debora MacKenzie Magazine issue 2284. 
 
FOUR years ago, Terry Singeltary watched his mother die horribly from a 
degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary 
was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded 
an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.
 
Most doctors believe that sCJD is caused by a prion protein deforming by 
chance into a killer. But Singeltary thinks otherwise. He is one of a number of 
campaigners who say that some sCJD, like the variant CJD related to BSE, is 
caused by eating meat from infected animals. Their suspicions have focused on 
sheep carrying scrapie, a BSE-like disease that is widespread in flocks across 
Europe and North America.
 
Now scientists in France have stumbled across new evidence that adds weight 
to the campaigners' fears. To their complete surprise, the researchers found 
that one strain of scrapie causes the same brain damage in mice as sCJD.
 
"This means we cannot rule out that at least some sCJD may be caused by 
some strains of scrapie," says team member Jean-Philippe Deslys of the French 
Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, 
south-west of Paris. Hans Kretschmar of the University of Göttingen, who 
coordinates CJD surveillance in Germany, is so concerned by the findings that he 
now wants to trawl back through past sCJD cases to see if any might have been 
caused by eating infected mutton or lamb.
 
Scrapie has been around for centuries and until now there has been no 
evidence that it poses a risk to human health. But if the French finding means 
that scrapie can cause sCJD in people, countries around the world may have 
overlooked a CJD crisis to rival that caused by BSE.
 
Deslys and colleagues were originally studying vCJD, not sCJD. They 
injected the brains of macaque monkeys with brain from BSE cattle, and from 
French and British vCJD patients. The brain damage and clinical symptoms in the 
monkeys were the same for all three. Mice injected with the original sets of 
brain tissue or with infected monkey brain also developed the same 
symptoms.
 
As a control experiment, the team also injected mice with brain tissue from 
people and animals with other prion diseases: a French case of sCJD; a French 
patient who caught sCJD from human-derived growth hormone; sheep with a French 
strain of scrapie; and mice carrying a prion derived from an American scrapie 
strain. As expected, they all affected the brain in a different way from BSE and 
vCJD. But while the American strain of scrapie caused different damage from 
sCJD, the French strain produced exactly the same pathology.
 
"The main evidence that scrapie does not affect humans has been 
epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute 
for Animal Health in Edinburgh, who was a member of the same team as Deslys. 
"You see about the same incidence of the disease everywhere, whether or not 
there are many sheep, and in countries such as New Zealand with no scrapie." In 
the only previous comparisons of sCJD and scrapie in mice, Bruce found they were 
dissimilar.
 
But there are more than 20 strains of scrapie, and six of sCJD. "You would 
not necessarily see a relationship between the two with epidemiology if only 
some strains affect only some people," says Deslys. Bruce is cautious about the 
mouse results, but agrees they require further investigation. Other trials of 
scrapie and sCJD in mice, she says, are in progress.
 
People can have three different genetic variations of the human prion 
protein, and each type of protein can fold up two different ways. Kretschmar has 
found that these six combinations correspond to six clinical types of sCJD: each 
type of normal prion produces a particular pathology when it spontaneously 
deforms to produce sCJD.
 
But if these proteins deform because of infection with a disease-causing 
prion, the relationship between pathology and prion type should be different, as 
it is in vCJD. "If we look at brain samples from sporadic CJD cases and find 
some that do not fit the pattern," says Kretschmar, "that could mean they were 
caused by infection."
 
There are 250 deaths per year from sCJD in the US, and a similar incidence 
elsewhere. Singeltary and other US activists think that some of these people 
died after eating contaminated meat or "nutritional" pills containing dried 
animal brain. Governments will have a hard time facing activists like Singeltary 
if it turns out that some sCJD isn't as spontaneous as doctors have 
insisted.
 
Deslys's work on macaques also provides further proof that the human 
disease vCJD is caused by BSE. And the experiments showed that vCJD is much more 
virulent to primates than BSE, even when injected into the bloodstream rather 
than the brain. This, says Deslys, means that there is an even bigger risk than 
we thought that vCJD can be passed from one patient to another through 
contaminated blood transfusions and surgical instruments.
 
 
Friday, January 30, 2015
 
*** Scrapie: a particularly persistent pathogen ***
 
 
Wednesday, December 24, 2014 
 
National Scrapie Eradication Program November 2014 Monthly Report Fiscal 
Year 2015 
 
 
Sunday, April 29, 2012 
 
Scrapie confirmed at quarantined sheep farm Canada CFIA 
 
 
Wednesday, April 4, 2012 
 
20120402 - Breach of quarantine/Violation de la mise en quarantaine of an 
ongoing Scrapie investigation 
 
 
Thursday, February 23, 2012 
 
Atypical Scrapie NOR-98 confirmed Alberta Canada sheep January 2012 
 
 
Thursday, March 29, 2012 
 
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012 
 
NIAA Annual Conference April 11-14, 2011San Antonio, Texas 
 
 
Monday, November 30, 2009 
 
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH 
CODE 
 
 
Friday, June 8, 2012 
 
Canadian Food Inspection Agency locates missing sheep 
 
 
Sunday, May 27, 2012 
 
CANADA PLANS TO IMPRISON ANYONE SPEAKING ABOUT MAD COW or ANY OTHER DISEASE 
OUTBREAK, CENSORSHIP IS A TERRIBLE THING 
 
 
EDMONTON - Some of former Alberta premier Ralph Klein's most colourful 
quotes — and the reactions they elicited: 
 
 SNIP
 
 "This all came about through the discovery of a single, isolated case of 
mad cow disease in one Alberta cow on May 20th. The farmer — I think he was a 
Louisiana fish farmer who knew nothing about cattle ranching. I guess any 
self-respecting rancher would have shot, shovelled and shut up, but he didn't do 
that." — Klein recalls how the mad cow crisis started and rancher Marwyn 
Peaster's role. The premier was speaking at the Western Governors Association 
meeting in Big Sky, Mont. September 2004. 
 
 "The premier meant that in an ironic or almost a sarcastic way." — Klein 
spokesman Gordon Turtle. 
 
 "You would have to eat 10 billion meals of brains, spinal cords, ganglia, 
eyeballs and tonsils." — Klein speaking in Montreal in January 2005 on the risk 
of humans contracting mad cow disease. 
 
 "I would offer $5 billion to have a Japanese person to come over here and 
eat nothing but Alberta beef for a year. And if he gets mad cow disease, I would 
be glad to give him $5 billion — make it $10 billion — Canadian." — Klein 
speaking after Japan closed its borders to Canadian beef. 
 
 
 
 Thursday, February 10, 2011 
 
 TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 
and how to hide mad cow disease in Canada Current as of: 2011-01-31 
 
 
 Wednesday, August 11, 2010 
 
 REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM 
ENCEPHALOPATHY (BSE) IN CANADA 
 
 
 Thursday, August 19, 2010 
 
 REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM 
ENCEPHALOPATHY (BSE) IN CANADA 
 
 
 Friday, March 4, 2011 
 
 Alberta dairy cow found with mad cow disease 
 
 
 Tuesday, May 21, 2013 
 
 Canada, USA, Bad feed, mad cows: Why we know three BSE cases had a common 
origin and why the SSS policy is in full force $$$ 
 
 
 Increased Atypical Scrapie Detections 
 
 Press reports indicate that increased surveillance is catching what 
otherwise would have been unreported findings of atypical scrapie in sheep. In 
2009, five new cases have been reported in Quebec, Ontario, Alberta, and 
Saskatchewan. With the exception of Quebec, all cases have been diagnosed as 
being the atypical form found in older animals. Canada encourages producers to 
join its voluntary surveillance program in order to gain scrapie-free status. 
The World Animal Health will not classify Canada as scrapie-free until no new 
cases are reported for seven years. The Canadian Sheep Federation is calling on 
the government to fund a wider surveillance program in order to establish the 
level of prevalence prior to setting an eradication date. Besides long-term 
testing, industry is calling for a compensation program for farmers who report 
unusual deaths in their flocks. 
 
 
 Current as of: 2015-01-31 
 
 Sheep flocks and/or goat herds confirmed to be infected with classical 
scrapie in Canada in 2015 Date confirmed Location Animal type infected January 5 
Ontario Goat 
 
 
 
 Tuesday, February 10, 2015 
 
 Alberta Canada First case of chronic wasting disease found in farm elk 
since 2002 
 
 
Wednesday, February 18, 2015 
 
OIE Bovine spongiform encephalopathy ,Canada 
 
 
Saturday, February 14, 2015 
 
Canadian Food Inspection Agency Confirms Bovine Spongiform Encephalopathy 
(BSE) in Alberta
 
 
Friday, February 20, 2015 
 
A BSE CANADIAN COW MAD COW UPDATE Transcript - Briefing (February 18, 2015) 
 
 
Monday, February 23, 2015 
 
20th BSE Case Raises New Concerns about Canada's Feeding Practices and 
Voluntary Testing Program; Highlights Importance of COOL 
 
 
Friday, February 20, 2015 
 
APHIS Freedom of Information Act (FOIA) Appeal Mouse Bio-Assays 
2007-00030-A Sheep Imported From Belgium and the Presence of TSE Prion Disease 
Kevin Shea to Singeltary 2015
 
 
Tuesday, February 17, 2015 
 
*** Could we spot the next BSE?, asks BVA President ***
 
 
Saturday, February 28, 2015
 
BSE CANADA UPDATE Transcript - Technical Briefing to Provide an Update on 
Investigation of Bovine Spongiform Encephalopathy in Alberta February 27, 2015 
4:00 p.m. 
 
 
Tuesday, February 10, 2015 
 
*** Alberta Canada First case of chronic wasting disease found in farm elk 
since 2002 
 
 
Wednesday, December 31, 2014 
 
NASDA BSE, CWD, SCRAPIE, TSE, PRION, Policy Statements updated with 
amendments passed during the NASDA Annual Meeting Updated September 18, 2014 
 
 
Sunday, December 28, 2014 
 
CHRONIC WASTING DISEASE CWD TSE PRION DISEASE AKA MAD DEER DISIEASE USDA 
USAHA INC DECEMBER 28, 2014 
 
 
Subject: *** Becky Lockhart 46, Utah’s first female House speaker, dies 
diagnosed with the extremely rare Creutzfeldt-Jakob disease aka mad cow type 
disease
 
what is CJD ? just ask USDA inc., and the OIE, they are still feeding the 
public and the media industry fed junk science that is 30 years old. 
 
why doesn’t some of you try reading the facts, instead of rubber stamping 
everything the USDA inc says.
 
sporadic CJD has now been linked to BSE aka mad cow disease, Scrapie, and 
there is much concern now for CWD and risk factor for humans. 
 
My sincere condolences to the family and friends of the House Speaker Becky 
Lockhart. I am deeply saddened hear this. 
 
with that said, with great respect, I must ask each and every one of you 
Politicians that are so deeply saddened to hear of this needless death of the 
Honorable House Speaker Becky Lockhart, really, cry me a friggen river. I am 
seriously going to ask you all this...I have been diplomatic for about 17 years 
and it has got no where. people are still dying. so, are you all stupid or 
what??? how many more need to die ??? how much is global trade of beef and other 
meat products that are not tested for the TSE prion disease, how much and how 
many bodies is this market worth?
 
Saturday, January 17, 2015 
 
*** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed 
with the extremely rare Creutzfeldt-Jakob disease
 
 
Thursday, January 15, 2015 
 
41-year-old Navy Commander with sporadic Creutzfeldt–Jakob disease CJD TSE 
Prion: Case Report 
 
 
*** HUMAN MAD COW DISEASE nvCJD TEXAS CASE NOT LINKED TO EUROPEAN TRAVEL 
CDC ***
 
Sunday, November 23, 2014 
 
*** Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas 
in June 2014 confirmed as USA case NOT European 
 
the patient had resided in Kuwait, Russia and Lebanon. The completed 
investigation did not support the patient's having had extended travel to 
European countries, including the United Kingdom, or travel to Saudi Arabia. The 
specific overseas country where this patient’s infection occurred is less clear 
largely because the investigation did not definitely link him to a country where 
other known vCJD cases likely had been infected. 
 
 
Sunday, December 14, 2014 
 
ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD 
strains, TSE prion aka Mad Cow Disease United States of America Update December 
14, 2014 Report
 
 
Sunday, February 08, 2015 
 
FDA SCIENCE BOARD TO THE FOOD AND DRUG ADMINISTRATION BOVINE HEPARIN BSE 
CJD TSE PRION Wednesday, June 4, 2014
 
 
Thursday, January 22, 2015 
 
Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to 
disease etiology?
 
 
Saturday, December 13, 2014 
 
Terry S. Singeltary Sr. Publications TSE prion disease 
 
Diagnosis and Reporting of Creutzfeldt-Jakob Disease 
 
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 
2001 JAMA 
 
snip... 
 
 
 
TSS
						 
						
						
					  
					  
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