Bad feed, mad cows: Why we know three BSE cases had a common origin
May 20, 2013. 2:27 pm • Section: Alberta, Business
In late 2003, days after a Canadian cow was found with bovine spongiform
encephalopathy south of the border, a Canadian Food Inspection Agency
investigator revealed to me he’d found a tentative link between that animal and
the one that, 10 years ago today, was confirmed as Canada’s first homegrown case
of BSE.
My paper at the time, the Edmonton Journal, put this on their front page on
Dec. 31. This bombshell finding was mentioned internationally, from Toronto to
Fox News to the New York Times. And the CFIA clammed up pretty quickly,
insisting this was merely a theory that hadn’t been proven.
By Jan. 23, 2006, nearly all of Canadian journalism had moved on from the
mad cow story. There was a much bigger story that day: the federal election that
would punt the Liberals and launch the Stephen Harper prime ministership.
That same day, the CFIA quietly published online an epidemiological report
on the roots of BSE. It confirmed that not only did Cow 1 and Cow 2 share a link
at the feed level, but so did a third mad cow.
Here’s my Edmonton Journal story on that 2006 report:
One day, one plant, three mad cows: On the third anniversary of Canada’s
BSE crisis, The Journal reveals how investigators traced the source of the
outbreak to a single day’s shipment of cattle protein from an Edmonton rendering
plant
Sat May 20 2006
EDMONTON – On Feb. 28, 1997, three loads of cattle protein left an Edmonton
rendering plant, destined for livestock feed mills in St. Paul, Edmonton and
Westlock.
The shipments were a perfectly normal event. Nobody would know it for
several years, but on that day the seed was planted for Canada’s mad cow
crisis.
The protein all three mills received contained the boiled-down remains of
an animal with mad cow disease that days or weeks earlier slipped, undetected,
past the inspection system, government investigators now believe.
Each of the three mills unwittingly turned those contaminated morsels into
cattle feed and each sold that feed to a farmer, who by giving it to his calves
infected one with the fatal, brain-wasting disease called bovine spongiform
encephalopathy, BSE. The practice of feeding cattle remains to cattle would be
banned months later to halt BSE, but was perfectly legal in early 1997.
Three cows, three farmers, three feed mills, one renderer, one day.
The shipments are now suspected as the origin of North America’s first
three cases of BSE found in homegrown cattle — including the initial case
announced in Alberta three years ago today. The discovery of that first mad cow
eventually choked off Canada’s global cattle trade and cost ranchers $7 billion
in lost sales.
The other two cases were an Alberta-born Holstein discovered in December
2003 in Washington state and a cow found near Barrhead in January 2005. All were
born between October 1996 and April 1997 in north-central Alberta or western
Saskatchewan, and probably ate feed made of material that came from the Edmonton
renderer that ultimately fateful day in February 1997.
This common link between the three mad cows is revealed in a report by the
Canadian Food Inspection Agency. Its detailed analysis raises some difficult,
controversial questions.
How many more mad cows or similar “clusters” are still out there? How many
other diseased cattle were never found, contaminating other cattle back in the
mid-1990s, when Canada tested a minute fraction of the number of cows it tests
for BSE today? Are Canada’s anti-BSE safeguards strict enough to protect
Canadians and the cattle supply? What does all this do to reassure consumers
that eating beef is still safe and mad cow is not widespread in Canada?
The report tries to clarify things by answering other nagging
queries.
“One of the questions that we’re always challenged to try to explain is,
‘Why Alberta? Why now? How did we get it?’ ” said Gary Little, a leading
veterinarian on the CFIA’s expansive mad cow file.
SHOTGUN SHELL ANALOGY
And what does this one-day link mean?
Think of a single shotgun shell, whose pellets spread to hit several
targets at once. The cluster points to one shotgun shell doing in all three
cows. According to this hypothesis, one mad cow, not two or three, infected
these cattle. And fewer shells fired could mean fewer potential hits
elsewhere.
The “geographic cluster” explains why BSE has been found mostly in Alberta
and not in Manitoba, Ontario or Quebec — although authorities will not rule out
finding future mad cows outside our province.
This cluster theory does not mean all cattle who ate feed originating from
that renderer’s shipments likely have BSE. The vast majority were either
slaughtered too young to get BSE or didn’t eat the diseased molecules. Among the
mad cows’ herdmates tested, none had BSE.
But the reported link only covers the first three Canadian BSE cases. There
have been three more since, and nothing the CFIA has found connects them to the
cluster traced to that day in February 1997.
One diseased animal, born in 1998 and discovered in January 2005, may have
been infected by feed-meal processed by a renderer in Calgary or Saskatoon, the
CFIA report says.
Two more cases that emerged earlier this year, both animals born in 2000,
were not covered in the report. One came from north-central Alberta, the other
was found last month in B.C.’s Fraser Valley, far from Canada’s existing BSE
loop.
The report on the B.C. mad cow hunt, expected out next week, will point to
tainted feed from several possible sources, including Alberta, Little
said.
“There’s a theory that would say, ‘Yeah, it’s likely the meat and bone meal
would have come from an Alberta plant, consistent with the other cases, gone to
B.C. and could have resulted in cross-contamination’ (with cattle feed),” he
said.
Gerald Ollis, Alberta’s chief veterinarian, hopes some link to this
province exists. Otherwise, “We have a new cluster” in Canada, he said, raising
the possibility of further cases in B.C.
“All I can do is hope they can trace it back to meat and bone meal from
this area,” Ollis said.
Ted Jacob/Herald
BAN’S SHORTCOMINGS REVEALED
The three cases outside the original cluster expose loopholes in the 1997
ban on cattle-to-cattle feeding, designed as the leading firewall against mad
cow. Authorities admit the ban will not eradicate BSE, since cattle remains can
still be used in feed for poultry, swine or other livestock that do not chew
cud. Traces of other feed can cross-contaminate cattle feed. The CFIA has
concluded at least one post-ban cow likely got sick because of improper
practices at one feed mill.
Only a miniscule amount of contaminated protein is enough to infect another
animal, European research has shown. Other countries, including the U.K., have
found BSE-infected animals born after a feed ban, although the number of cases
had dropped significantly.
To stop this cross-contamination, the CFIA proposed in December 2004 to
expand the feed ban to exclude BSE-prone cattle parts — brains, spine and other
“high-risk” tissues — from all animal feed. The rule remains unimposed 11/2
years later, while cattle-industry groups worry about the disposal costs and
environmental effects of tonnes of waste.
While conceding that BSE cases may still emerge, disease experts insist
consumers are still safe. In 2003, Canada began removing high-risk cattle parts
from the human food chain. This is internationally accepted as one of the best
ways to prevent the human form of mad cow, variant Creutzfeldt-Jakob
disease.
Little admits the CFIA will never know how many BSE cattle went undetected
before the first discovered case in 2003. But Canadian slaughterhouse inspectors
have for decades barred sickly cattle from being killed for meat, Ollis
said.
By not taking more decisive early action, Canada did put its animals and
citizens at risk, argues William Leiss, a University of Ottawa scientist and
author of Mad Cows and Mother’s Milk.
Because Canada tested so few animals in the 1990s, unknown numbers of
diseased cattle slipped through the system then — the “first generation” of
homegrown mad cows — and were processed into feed that infected the “second
generation”of BSE cases since 2003, Leiss said.
“We have no idea how much infectivity there was in the first generation (of
Canadian BSE) because we had such lousy surveillance,” he said. “Our
surveillance stank. We didn’t want to know.”
Leiss has long pushed for tighter feed controls and more testing, and still
believes stronger measures, like an enhanced feed ban, are needed.
The CFIA argues the steps Canada took in the 1990s met and exceeded
international targets for countries without homegrown BSE, and current
regulations now surpass expectations for countries with BSE. Little also said
the post-feed-ban cases underline the need for a tougher ban.
The hunt for BSE origins in Canada stretches back nearly two decades and
has featured some of the most elaborate sleuthing in CFIA history, from sifting
through 1980s farm-health records in Britain to checking sales receipts in
Alberta farms and mills. The exhaustive search wound up connecting three mad
cows to one day’s rendering plant shipments.
NOW FOUND IN 23 NATIONS
BSE, first discovered in British cattle in 1986, has turned up in 23
countries, including Canada, the U.S. and Japan. International scientific
consensus holds that BSE is spread when cattle eat the high-risk materials of
infected cattle.
In 1990, as the disease was becoming a crisis overseas, Canada banned
imports of live cattle from Britain.
When one of the British cows on a farm near Red Deer tested positive for
BSE in 1993, the CFIA tracked the other 167 cattle imported from the U.K. since
1982. It destroyed and tested all those still alive, and none had BSE. But 68 of
the imported cattle had already been slaughtered or died naturally, nearly half
of them in Alberta.
Investigators contacted Britain for farm histories of those dead British
animals. Ten became classified as “high risk” because they came from farms that
reported other BSE cases. One died in Quebec, two in Ontario, seven in
Alberta.
The CFIA’s hypothesis contends that at least one of those 68 cattle had the
disease, and died in the early 1990s, likely in Alberta. Its carcass was boiled
down at a rendering plant, which sent its protein to dozens of feed mills, where
it was processed for cattle feed long before the BSE feed ban was imposed.
That protein infected one or more Canadian-born animals, and since BSE is
known to normally incubate for six years or more before symptoms emerge, those
diseased animals died around 1996 or 1997. They were never detected, at a time
when mere hundreds of cows were tested each year for BSE among the more than
three million cattle slaughtered. Now, authorities test about 5,000 cattle a
month, focussing on the ones most likely to have BSE: aging cattle that are
diseased, “downers” or dead.
One of these animals was killed or died in north-central Alberta, and its
remains would have been shipped to Northern Alberta Processing Co. in Edmonton,
the only rendering plant in Alberta north of Calgary. This happened days or
weeks
before Feb. 28, 1997.
Fast-forward to recent years, and the ranches where the latest BSE cases
were born. CFIA investigators pored over farmers’ years-old purchasing records,
queried their feeding practices, and pinpointed feed containing protein meal the
mad cows had eaten as calves. Some farmers kept complete records and sales
receipts, but there were gaps, which made the tracing difficult, Little
said.
Investigators then went to mills where suspect feed had been bought, in
most cases multiple mills per mad cow. They discovered what ingredients were in
each feed, and learned which renderers the mills bought from. When the trail led
to Northern Alberta Processing, shipping manifests helped lock in the final
puzzle pieces, leading to the theory of consecutive shipments in February
1997.
While the one-day link is compelling, the federal veterinarian cautions
that it cannot be confirmed. It is impossible to determine exactly what a cow
ate several years ago, and there is none of the old feed left to be tested. The
report can at best pose a hypothesis and draw links, experts insist.
Alberta might continue to find mad cows because of the disease’s history
and the province’s testing program, Ollis said. Alberta ranchers get $225 for
each bovine head surrendered for government testing, the richest incentive in
Canada. The province performs more than half of Canada’s BSE tests.
“The enhanced level of BSE surveillance in Alberta set us up for finding a
cluster,” Ollis said. “Obviously it’s here, so we want to know how much of it is
here.”
The CFIA cannot pinpoint how many future mad cows will emerge, but has
adopted a promising new tool — a prediction model developed by scientists in
Europe and given preliminary praise by the European Food Safety Authority.
The BSurvE model processed data on surveillance statistics, the feed ban,
cattle population and other factors. It predicted three more mad cows in Canada
beyond the ones already discovered at the time the CFIA report was written. “The
model also predicted that these infected cattle would have been born before or
during the implementation of the 1997 feed ban,” the report says.
On Jan. 23, 2006, the same day the report was made public, CFIA announced
the discovery of a mad cow in north-central Alberta that had been born in 2000 —
three years after Canada’s feed ban and contrary to the model’s prediction. Then
in April, another mad cow was found in B.C.
This does not mean that, based on the BSurvE model, inspectors will only
find one more mad cow, Little said. Rather, it shows the BSurvE model is not
perfect.
But since it did not predict several dozen or hundreds of diseased cattle
lurking out there to be discovered, it should give comfort to Canadian
consumers. And based on European history, Canada’s existing feed ban, even with
its loopholes, will eventually reduce mad cow cases to close to zero.
“Probably more important than the number three is the fact it shows that
based on the information we have, BSE is a very, very rare event in this
country,” Little said of the BSurvE model. “Whether it’s three (cases) or it’s
10 is probably not as important.”
on the 10 year anniversary of mad cow disease in Canada, your still just
kidding yourself, your readers, and at the same time, putting everyone at risk
by this fallacy. ...tss
CFIA, USDA, AND OIE SHOOT, SHOVEL, AND SHUT THE HELL UP SSS BSE TSE PRION
MAD COW TYPE POLICY $$$, and the media is buying it hook, line, and sinker
$$$
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.
Wednesday, December 22, 2010.
Manitoba veterinarian has been fined $10,000 for falsifying certification
documents for U.S. bound cattle and what about mad cow disease?
CENSORSHIP IS A TERRIBLE THING $$$.
Canada has had a COVER-UP policy of mad cow disease since about the 17th
case OR 18th case of mad cow disease. AFTER THAT, all FOIA request were ignored
$$$.
THIS proves there is indeed an epidemic of mad cow disease in North
America, and it has been covered up for years and years, if not for decades, and
it’s getting worse $$$.
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.
Thursday, January 17, 2013.
Canada, U.S. agree on animal-disease measures to protect trade, while
reducing human and animal health protection.
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.
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.
please note, I do not know how much of this 125 TONS of banned mad cow
protein was part of the ;
e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;
bbbut, this was about 10 years post mad cow feed ban from 1997. 10 years
later, and still feeding banned mad cow protein to cervids???
considering that .005 gram is lethal to several bovines, and we know that
the oral consumption of CWD tainted products is very efficient mode of
transmission of CWD.
Subject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125
TONS Products manufactured from 02/01/2005 until 06/06/2006
Date: August 6, 2006 at 6:16 pm PST
PRODUCT
a) CO-OP 32% Sinking Catfish, Recall # V-100-6;
b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall #
V-101-6;
c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6;
d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6;
***e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;
f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50
lb. bag, Recall # V-105-6;
g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%,
Recall # V-106-6;
h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to
20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall #
V-107-6;
i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall # 108-6;
j) CO-OP LAYING CRUMBLES, Recall # V-109-6;
k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall #
V-110-6;
l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6;
m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6
CODE
Product manufactured from 02/01/2005 until 06/06/2006
RECALLING FIRM/MANUFACTURER
Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email
and visit on June 9, 2006. FDA initiated recall is complete.
REASON
Animal and fish feeds which were possibly contaminated with ruminant based
protein not labeled as "Do not feed to ruminants".
VOLUME OF PRODUCT IN COMMERCE
125 tons
DISTRIBUTION
AL and FL
END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006
###
*** 10,000,000 lbs banned blood laced meat and bone meal mbm 2007 one
decade post partial and voluntary mad cow feed ban was put in place ;
snip...see more here ;
Saturday, August 4, 2012
Final Feed Investigation Summary - California BSE Case - July 2012
SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE
INVESTIGATION JULY 2012
Summary Report BSE 2012
Executive Summary
Saturday, August 4, 2012
Update from APHIS Regarding Release of the Final Report on the BSE
Epidemiological Investigation
Saturday, December 15, 2012
Bovine spongiform encephalopathy: the effect of oral exposure dose on
attack rate and incubation period in cattle -- an update 5 December 2012
Saturday, December 15, 2012
Bovine spongiform encephalopathy: the effect of oral exposure dose on
attack rate and incubation period in cattle -- an update 5 December 2012
Friday, April 19, 2013
FDA BSE TSE PRION NEWS FEED AND ANNUAL INSPECTION OF FEED MILLS REPORTS HAS
CEASED TO EXIST
Monday, March 25, 2013
Minnesota Firm Recalls Bone-In Ribeye That May Contain Specified Risk
Materials Recall Release CLASS II RECALL FSIS-RC-024-2013
Friday, April 19, 2013.
APHIS 2013 Stakeholder Meeting (March 2013) BSE TSE PRION.
Monday, October 10, 2011.
EFSA Journal 2011 The European Response to BSE: A Success Story.
snip...
EFSA and the European Centre for Disease Prevention and Control (ECDC)
recently delivered a scientific opinion on any possible epidemiological or
molecular association between TSEs in animals and humans (EFSA Panel on
Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical
BSE prions as the only TSE agents demonstrated to be zoonotic so far but the
possibility that a small proportion of human cases so far classified as
"sporadic" CJD are of zoonotic origin could not be excluded. Moreover,
transmission experiments to non-human primates suggest that some TSE agents in
addition to Classical BSE prions in cattle (namely L-type Atypical BSE,
Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic
wasting disease (CWD) agents) might have zoonotic potential.
snip...
Thursday, August 12, 2010.
Seven main threats for the future linked to prions.
First threat
The TSE road map defining the evolution of European policy for protection
against prion diseases is based on a certain numbers of hypotheses some of which
may turn out to be erroneous. In particular, a form of BSE (called atypical
Bovine Spongiform Encephalopathy), recently identified by systematic testing in
aged cattle without clinical signs, may be the origin of classical BSE and thus
potentially constitute a reservoir, which may be impossible to eradicate if a
sporadic origin is confirmed.
***Also, a link is suspected between atypical BSE and some apparently
sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases
constitute an unforeseen first threat that could sharply modify the European
approach to prion diseases.
Second threat
snip...
Sunday, February 10, 2013
Creutzfeldt-Jakob disease (CJD) biannual update (February 2013) Infection
report/CJD
Tuesday, March 5, 2013
Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening
of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION)
FDA believes current regulation protects the public from BSE but reopens
comment period due to new studies
Tuesday, March 05, 2013
A closer look at prion strains Characterization and important implications
Prion
7:2, 99–108; March/April 2013; © 2013 Landes Bioscience
Wednesday, March 20, 2013
GAO-13-244, Mar 18, 2013 Dietary Supplements FDA May Have Opportunities to
Expand Its Use of Reported Health Problems to Oversee Product
From: Terry S. Singeltary Sr.
Sent: Tuesday, March 19, 2013 2:46 PM
To: gomezj@gao.gov
Cc: siggerudk@gao.gov ; youngc1@gao.gov ; oighotline@gao.gov
Wednesday, February 20, 2013
World Organization for Animal Health Recommends United States' BSE Risk
Status Be Upgraded
Statement from Agriculture Secretary Tom Vilsack:
Thursday, February 14, 2013
*** The Many Faces of Mad Cow Disease Bovine Spongiform Encephalopathy BSE
and TSE prion disease
Thursday, February 21, 2013
National Prion Disease Pathology Surveillance Center Cases Examined January
16, 2013
16 YEAR OLD SPORADIC FFI ?
Monday, January 14, 2013
Gambetti et al USA Prion Unit change another highly suspect USA mad cow
victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes
along with this BSe
Monday, December 31, 2012
Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State,
2006–2011-2012
Tuesday, December 25, 2012
CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25,
2012
Tuesday, June 26, 2012
*** Creutzfeldt Jakob Disease Human TSE report update North America,
Canada, Mexico, and USDA PRION UNIT as of May 18, 2012
type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the
rise in Canada and the USA
Wednesday, June 13, 2012
MEXICO IS UNDER or MIS DIAGNOSING CREUTZFELDT JAKOB DISEASE AND OTHER PRION
DISEASE SOME WITH POSSIBLE nvCJD
*** The discovery of previously unrecognized prion diseases in both humans
and animals (i.e., Nor98 in small ruminants) demonstrates that the range of
prion diseases might be wider than expected and raises crucial questions about
the epidemiology and strain properties of these new forms. We are investigating
this latter issue by molecular and biological comparison of VPSPr, GSS and
Nor98.
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion
poker goes up again $
OR-10: Variably protease-sensitive prionopathy is transmissible in bank
voles
Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1
Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan
Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome,
Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna,
Italy; 3Case Western Reserve University; Cleveland, OH USA
Background. Variably protease-sensitive prionopathy (VPSPr) is a recently
described “sporadic”neurodegenerative disease involving prion protein
aggregation, which has clinical similarities with non-Alzheimer dementias, such
as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in
Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the
prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is
the electrophoretic pattern of PrPSc after digestion with proteinase K (PK).
After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern
similar to that described in GSS cases. The clinical and pathological features
of VPSPr raised the question of the correct classification of VPSPr among prion
diseases or other forms of neurodegenerative disorders. Here we report
preliminary data on the transmissibility and pathological features of VPSPr
cases in bank voles.
Materials and Methods. Seven VPSPr cases were inoculated in two genetic
lines of bank voles, carrying either methionine or isoleucine at codon 109 of
the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases
selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical
diagnosis in voles was confirmed by brain pathological assessment and western
blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.
Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission
in BvM109. Overall, 3 voles were positive with survival time between 290 and 588
d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form
of the typical PrP27–30, which was indistinguishable to that previously observed
in BvM109 inoculated with sCJDMM1 cases.
In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until
now. Overall, 5 voles were positive with survival time between 281 and 596
d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like
PrPSc electrophoretic pattern, characterized by low molecular weight PrPres.
These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative
with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus
and the N-terminus. Second passages are in progress from these first successful
transmissions.
Conclusions. Preliminary results from transmission studies in bank voles
strongly support the notion that VPSPr is a transmissible prion disease.
Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of
voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.
The discovery of previously unrecognized prion diseases in both humans and
animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion
diseases might be wider than expected and raises crucial questions about the
epidemiology and strain properties of these new forms. We are investigating this
latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.
Wednesday, March 28, 2012
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion
poker goes up again $
Sunday, March 31, 2013
Creutzfeldt Jakob Disease CJD worlds youngest documented victim, 11 years
old, shall we pray
Monday, April 15, 2013
Dr. Stephen B. Thacker Director Centers for Disease Control and
Prevention′s Office of Science, Epidemiology and Laboratory Services (OSELS)
dies from Creutzfeldt Jakob Disease CJD
TSS
