Canada Transmissible Spongiform Encephalopathy TSE Prion Report Update
Canada Transmissible Spongiform
Encephalopathy TSE Prion Report Update
Domestic cervid herds confirmed to
be infected with CWD in Canada in 2016
Current as of: 2016-10-31
Domestic cervid herds confirmed to
be infected with CWD in Canada in 2016
Date confirmed Location Animal type
infected
October 3 Alberta Elk
February 18 Saskatchewan Deer
March 21 Saskatchewan Deer
May 18 Saskatchewan Elk
July 21 Saskatchewan Elk
Scrapie Canada
Current as of: 2016-05-31
Sheep flocks and/or goat herds
confirmed to be infected with classical scrapie in Canada in 2016
Date confirmed
Location
Animal type infected
May 18
Ontario
Goat
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.
Thursday, February 23, 2012
Atypical Scrapie NOR-98 confirmed
Alberta Canada sheep January 2012
Thursday, October 15, 2009
SCRAPIE UPDATE CANADA 2009 (typical
and atypical cases)
Bovine Spongiform Encephalopathy
BSE
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.
Monday, February 23, 2015
20th BSE Case Raises New Concerns
about Canada's Feeding Practices and Voluntary Testing Program; Highlights
Importance of COOL
Saturday, February 14, 2015
Canadian Food Inspection Agency
Confirms Bovine Spongiform Encephalopathy (BSE) in Alberta
Friday, January 10, 2014
USDA AUDIT ON CANADA'S MEAT
INSPECTION DISTURBING (pot calling kettle black again)
US audit finds Canada's meat
inspections wanting
USDA AUDIT ON CANADA'S MEAT
INSPECTION DISTURBING (pot calling kettle black again)
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.
---
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 $$$.
Thursday, February 10, 2011.
TRANSMISSIBLE SPONGIFORM
ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow
disease in Canada Current as of: 2011-01-31.
Thursday, January 17, 2013.
Canada, U.S. agree on
animal-disease measures to protect trade, while reducing human and animal
health protection.
Wednesday, August 11, 2010.
REPORT ON THE INVESTIGATION OF THE
SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA.
Thursday, August 19, 2010.
REPORT ON THE INVESTIGATION OF THE
SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA.
Friday, March 4, 2011.
Alberta dairy cow found with mad
cow disease.
Tuesday, May 21, 2013
Canada, USA, Bad feed, mad cows:
Why we know three BSE cases had a common origin and why the SSS policy is in
full force $$$
Docket No. 03-080-1 -- USDA ISSUES
PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA http://madcowfeed.blogspot.com/2008/07/docket-no-03-080-1-usda-issues-proposed.html
CANADA MBM LIVE CATTLE BSE TSE
PRION TO USA
Date: Sat, 14 Jun 2003 02:23:12
+0200
OIG REPORT ON IMPORTS FROM CANADA
Tuesday, October 2, 2012
Canadian veterinarian fined after
approving banned BSE high risk cattle for export to U.S.A.
Saturday, January 21, 2012
Quick facts about mad cow disease
Thursday, January 17, 2013
Canada, U.S. agree on
animal-disease measures to protect trade, while reducing human and animal
health protection
Sunday, December 2, 2012
CANADA 19 cases of mad cow disease
SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’
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
2009 UPDATE ON ALABAMA AND TEXAS
MAD COWS 2005 and 2006
Wednesday, December 4, 2013
*** Bovine Spongiform
Encephalopathy; Importation of Bovines and Bovine Products; Final Rule Federal
Register / Vol. 78 , No. 233 / Wednesday, December 4, 2013
Saturday, November 2, 2013
*** APHIS Finalizes Bovine Import
Regulations in Line with International Animal Health Standards while enhancing
the spread of BSE TSE prion mad cow type disease around the Globe
Monday, November 4, 2013
*** R-CALF Bullard new BSE rule
represents the abrogation of USDA’s responsibility to protect U.S. consumers
and the U.S. cattle herd from the introduction of foreign animal disease ***
Wednesday, October 30, 2013
SPECIFIED RISK MATERIAL (SRM)
CONTROL VERIFICATION TASK FSIS NOTICE 70-13 10/30/13
Bovine Spongiform Encephalopathy;
Minimal-Risk Regions; Importation of Live Bovines and Products Derived from
Bovines Commodities APHIS-2006-0041
*** 2016 ***
Tuesday, July 26, 2016
*** Atypical Bovine Spongiform
Encephalopathy BSE TSE Prion UPDATE JULY 2016 ***
Saturday, July 23, 2016
BOVINE SPONGIFORM ENCEPHALOPATHY
BSE TSE PRION SURVEILLANCE, TESTING, AND SRM REMOVAL UNITED STATE OF AMERICA
UPDATE JULY 2016
Wednesday, November 09, 2016
Chronic Wasting Disease (CWD)
Program Standards - Review and Comment By Terry S Singeltary Sr. November 9,
2016
Saturday, December 12, 2015
NOTICE: Environmental Impact
Statement on Large Livestock Carcasses TSE Prion REPORT December 14, 2015
Friday, August 14, 2015
Carcass Management During a Mass
Animal Health Emergency Draft Programmatic Environmental Impact
Statement—August 2015
Wednesday, September 7, 2016
An assessment of the long-term
persistence of prion infectivity in aquatic environments
***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.***
***at present, no cervid PrP allele
conferring absolute resistance to prion infection has been identified.
P-145 Estimating chronic wasting
disease resistance in cervids using real time quaking- induced conversion
Nicholas J Haley1, Rachel
Rielinqer2, Kristen A Davenport3, W. David Walter4, Katherine I O'Rourke5,
Gordon Mitchell6, Juergen A Richt2
1 Department of Microbiology and
Immunology, Midwestern University, United States; 2Department of Diagnostic
Medicine and Pathobiology, Kansas State University; 3Prion Research Center;
Colorado State University; 4U.S. Geological Survey, Pennsylvania Cooperative
Fish and Wildlife Research Unit; 5Agricultural Research Service, United States
Department of Agriculture; 6Canadian Food Inspection Agency, National and OlE
Reference Laboratory for Scrapie and CWD
In mammalian species, the
susceptibility to prion diseases is affected, in part, by the sequence of the
host's prion protein (PrP). In sheep, a gradation from scrapie susceptible to
resistant has been established both in vivo and in vitro based on the amino
acids present at PrP positions 136, 154, and 171, which has led to global
breeding programs to reduce the prevalence of scrapie in domestic sheep. In
cervids, resistance is commonly characterized as a delayed progression of
chronic wasting disease (CWD); at present, no cervid PrP allele conferring
absolute resistance to prion infection has been identified. To model the
susceptibility of various naturally-occurring and hypothetical cervid PrP
alleles in vitro, we compared the amplification rates and efficiency of various
CWD isolates in recombinant PrPC using real time quaking-induced conversion. We
hypothesized that amplification metrics of these isolates in cervid PrP substrates
would correlate to in vivo susceptibility - allowing susceptibility prediction
for alleles found at 10 frequency in nature, and that there would be an
additive effect of multiple resistant codons in hypothetical alleles. Our
studies demonstrate that in vitro amplification metrics predict in vivo
susceptibility, and that alleles with multiple codons, each influencing
resistance independently, do not necessarily contribute additively to
resistance. Importantly, we found that the white-tailed deer 226K substrate
exhibited the slowest amplification rate among those evaluated, suggesting that
further investigation of this allele and its resistance in vivo are warranted
to determine if absolute resistance to CWD is possible.
***at present, no cervid PrP allele
conferring absolute resistance to prion infection has been identified.
PRION 2016 CONFERENCE TOKYO
PRION 2016 TOKYO
*** Zoonotic Potential of CWD
Prions: An Update ***
Ignazio Cali1, Liuting Qing1, Jue
Yuan1, Shenghai Huang2, Diane Kofskey1,3, Nicholas Maurer1, Debbie McKenzie4,
Jiri Safar1,3,5, Wenquan Zou1,3,5,6, Pierluigi Gambetti1, Qingzhong Kong1,5,6
1Department of Pathology, 3National Prion Disease Pathology Surveillance
Center, 5Department of Neurology, 6National Center for Regenerative Medicine,
Case Western Reserve University, Cleveland, OH 44106, USA. 4Department of
Biological Sciences and Center for Prions and Protein Folding Diseases, University
of Alberta, Edmonton, Alberta, Canada, 2Encore Health Resources, 1331 Lamar St,
Houston, TX 77010
Chronic wasting disease (CWD) is a
widespread and highly transmissible prion disease in free-ranging and captive
cervid species in North America. The zoonotic potential of CWD prions is a
serious public health concern, but the susceptibility of human CNS and
peripheral organs to CWD prions remains largely unresolved. We reported earlier
that peripheral and CNS infections were detected in transgenic mice expressing
human PrP129M or PrP129V. Here we will present an update on this project,
including evidence for strain dependence and influence of cervid PrP
polymorphisms on CWD zoonosis as well as the characteristics of experimental
human CWD prions.
PRION 2016 TOKYO In Conjunction
with Asia Pacific Prion Symposium 2016 PRION 2016 Tokyo Prion 2016
Prion 2016 Purchase options Price *
Issue Purchase USD 198.00
http://www.tandfonline.com/toc/kprn20/10/sup1
Cervid to human prion transmission
Kong, Qingzhong
Case Western Reserve University,
Cleveland, OH, United States
Abstract
Prion disease is transmissible and
invariably fatal. Chronic wasting disease (CWD) is the prion disease affecting
deer, elk and moose, and it is a widespread and expanding epidemic affecting 22
US States and 2 Canadian provinces so far. CWD poses the most serious zoonotic
prion transmission risks in North America because of huge venison consumption
(>6 million deer/elk hunted and consumed annually in the USA alone),
significant prion infectivity in muscles and other tissues/fluids from
CWD-affected cervids, and usually high levels of individual exposure to CWD
resulting from consumption of the affected animal among often just family and
friends. However, we still do not know whether CWD prions can infect humans in
the brain or peripheral tissues or whether clinical/asymptomatic CWD zoonosis
has already occurred, and we have no essays to reliably detect CWD infection in
humans. We hypothesize that:
(1) The classic CWD prion strain
can infect humans at low levels in the brain and peripheral lymphoid tissues;
(2) The cervid-to-human
transmission barrier is dependent on the cervid prion strain and influenced by
the host (human) prion protein (PrP) primary sequence;
(3) Reliable essays can be
established to detect CWD infection in humans;and
(4) CWD transmission to humans has
already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg)
mouse models and complementary in vitro approaches.
Aim 1 will prove that the classical
CWD strain may infect humans in brain or peripheral lymphoid tissues at low
levels by conducting systemic bioassays in a set of "humanized" Tg
mouse lines expressing common human PrP variants using a number of CWD isolates
at varying doses and routes. Experimental "human CWD" samples will
also be generated for Aim 3.
Aim 2 will test the hypothesis that
the cervid-to-human prion transmission barrier is dependent on prion strain and
influenced by the host (human) PrP sequence by examining and comparing the
transmission efficiency and phenotypes of several atypical/unusual CWD isolates/strains
as well as a few prion strains from other species that have adapted to cervid
PrP sequence, utilizing the same panel of humanized Tg mouse lines as in Aim 1.
Aim 3 will establish reliable
essays for detection and surveillance of CWD infection in humans by examining
in details the clinical, pathological, biochemical and in vitro seeding
properties of existing and future experimental "human CWD" samples
generated from Aims 1-2 and compare them with those of common sporadic human Creutzfeldt-Jakob
disease (sCJD) prions.
Aim 4 will attempt to detect
clinical CWD-affected human cases by examining a significant number of brain
samples from prion-affected human subjects in the USA and Canada who have
consumed venison from CWD-endemic areas utilizing the criteria and essays
established in Aim 3. The findings from this proposal will greatly advance our
understandings on the potential and characteristics of cervid prion
transmission in humans, establish reliable essays for CWD zoonosis and
potentially discover the first case(s) of CWD infection in humans.
Public Health Relevance There are
significant and increasing human exposure to cervid prions because chronic
wasting disease (CWD, a widespread and highly infectious prion disease among
deer and elk in North America) continues spreading and consumption of venison
remains popular, but our understanding on cervid-to-human prion transmission is
still very limited, raising public health concerns. This proposal aims to
define the zoonotic risks of cervid prions and set up and apply essays to
detect CWD zoonosis using mouse models and in vitro methods. The findings will
greatly expand our knowledge on the potentials and characteristics of cervid
prion transmission in humans, establish reliable essays for such infections and
may discover the first case(s) of CWD infection in humans.
Funding Agency Agency National
Institute of Health (NIH)
Institute National Institute of
Neurological Disorders and Stroke (NINDS)
Type Research Project (R01)
Project # 1R01NS088604-01A1
Application # 9037884
Study Section Cellular and
Molecular Biology of Neurodegeneration Study Section (CMND)
Program Officer Wong, May
Project Start 2015-09-30
Project End 2019-07-31
Budget Start 2015-09-30
Budget End 2016-07-31
Support Year 1
Fiscal Year 2015
Total Cost $337,507
Indirect Cost $118,756
Institution
Name Case Western Reserve
University
Department Pathology
Type Schools of Medicine
DUNS # 077758407
City Cleveland
State OH
Country United States
Zip Code 44106
===========================================================
We hypothesize that:
(1) The classic CWD prion strain
can infect humans at low levels in the brain and peripheral lymphoid tissues;
(2) The cervid-to-human
transmission barrier is dependent on the cervid prion strain and influenced by
the host (human) prion protein (PrP) primary sequence;
(3) Reliable essays can be
established to detect CWD infection in humans;and
(4) *** CWD transmission to humans
has already occurred. *** We will test these hypotheses in 4 Aims using
transgenic (Tg) mouse models and complementary in vitro approaches.
============================================================
Key Molecular Mechanisms of TSEs
Zabel, Mark D.
Colorado State University-Fort
Collins, Fort Collins, CO, United States
Abstract Prion diseases, or
transmissible spongiform encephalopathies (TSEs), are fatal neurodegenerative
diseases affecting humans, cervids, bovids, and ovids. The absolute requirement
of PrPC expression to generate prion diseases and the lack of instructional
nucleic acid define prions as unique infectious agents. Prions exhibit
species-specific tropism, inferring that unique prion strains exist that
preferentially infct certain host species and confront transmission barriers to
heterologous host species. However, transmission barriers are not absolute.
Scientific consensus agrees that the sheep TSE scrapie probably breached the
transmission barrier to cattle causing bovine spongiform encephalopathy that
subsequently breached the human transmission barrier and likely caused several
hundred deaths by a new-variant form of the human TSE Creutzfeldt-Jakob disease
in the UK and Europe. The impact to human health, emotion and economies can
still be felt in areas like farming, blood and organ donations and the threat
of a latent TSE epidemic. This precedent raises the real possibility of other
TSEs, like chronic wasting disease of cervids, overcoming similar human
transmission barriers. A groundbreaking discovery made last year revealed that
mice infected with heterologous prion strains facing significant transmission
barriers replicated prions far more readily in spleens than brains6.
Furthermore, these splenic prions exhibited weakened transmission barriers and
expanded host ranges compared to neurogenic prions. These data question
conventional wisdom of avoiding neural tissue to avoid prion xenotransmission,
when more promiscuous prions may lurk in extraneural tissues. Data derived from
work previously funded by NIH demonstrate that Complement receptors CD21/35
bind prions and high density PrPC and differentially impact prion disease
depending on the prion isolate or strain used. Recent advances in live animal
and whole organ imaging have led us to generate preliminary data to support
novel, innovative approaches to assessing prion capture and transport. We plan
to test our unifying hypothesis for this proposal that CD21/35 control the
processes of peripheral prion capture, transport, strain selection and
xenotransmission in the following specific aims. 1. Assess the role of CD21/35
in splenic prion strain selection and host range expansion. 2. Determine
whether CD21/35 and C1q differentially bind distinct prion strains 3. Monitor
the effects of CD21/35 on prion trafficking in real time and space 4. Assess
the role of CD21/35 in incunabular prion trafficking
Public Health Relevance Transmissible
spongiform encephalopathies, or prion diseases, are devastating illnesses that
greatly impact public health, agriculture and wildlife in North America and
around the world. The impact to human health, emotion and economies can still
be felt in areas like farming, blood and organ donations and the threat of a
latent TSE epidemic. This precedent raises the real possibility of other TSEs,
like chronic wasting disease (CWD) of cervids, overcoming similar human
transmission barriers. Early this year Canada reported its first case of BSE in
over a decade audits first case of CWD in farmed elk in three years,
underscoring the need for continued vigilance and research. Identifying
mechanisms of transmission and zoonoses remains an extremely important and intense
area of research that will benefit human and other animal populations.
Funding Agency Agency National
Institute of Health (NIH)
Institute National Institute of
Allergy and Infectious Diseases (NIAID)
Type High Priority, Short Term
Project Award (R56)
Project # 1R56AI122273-01A1
Application # 9211114
Study Section Cellular and
Molecular Biology of Neurodegeneration Study Section (CMND)
Program Officer Beisel, Christopher
E
Project Start 2016-02-16
Project End 2017-01-31
Budget Start 2016-02-16
Budget End 2017-01-31
Support Year 1
Fiscal Year 2016
Total Cost
Indirect Cost Institution Name
Colorado State University-Fort Collins
Department
Microbiology/Immun/Virology
Type Schools of Veterinary Medicine
DUNS # 785979618 City Fort Collins
State CO
Country United States
Zip Code 80523
PMCA Detection of CWD Infection in
Cervid and Non-Cervid Species
Hoover, Edward Arthur
Colorado State University-Fort
Collins, Fort Collins, CO, United States
Abstract Chronic wasting disease
(CWD) of deer and elk is an emerging highly transmissible prion disease now
recognized in 18 States, 2 Canadian provinces, and Korea. We have shown that
Infected deer harbor and shed high levels of infectious prions in saliva,
blood, urine, and feces, and in the tissues generating those body fluids and
excreta, thereby leading to facile transmission by direct contact and environmental
contamination. We have also shown that CWD can infect some non-cervid species,
thus the potential risk CWD represents to domestic animal species and to humans
remains unknown. Whether prions borne in blood, saliva, nasal fluids, milk, or
excreta are generated or modified in the proximate peripheral tissue sites, may
differ in subtle ways from those generated in brain, or may be adapted for
mucosal infection remain open questions. The increasing parallels in the
pathogenesis between prion diseases and human neurodegenerative conditions,
such as Alzheimer's and Parkinson's diseases, add relevance to CWD as a
transmissible protein misfolding disease. The overall goal of this work is to
elucidate the process of CWD prion transmission from mucosal secretory and
excretory tissue sites by addressing these questions: (a) What are the kinetics
and magnitude of CWD prion shedding post-exposure? (b) Are excreted prions
biochemically distinct, or not, from those in the CNS? (c) Are peripheral
epithelial or CNS tissues, or both, the source of excreted prions? and (d) Are
excreted prions adapted for horizontal transmission via natural/trans-mucosal
routes? The specific aims of this proposal are: (1) To determine the onset and
consistency of CWD prion shedding in deer and cervidized mice; (2); To compare
the biochemical and biophysical properties of excretory vs. CNS prions; (3) To
determine the capacity of peripheral tissues to support replication of CWD
prions; (4) To determine the protease- sensitive infectious fraction of
excreted vs. CNS prions; and (5) To compare the mucosal infectivity of
excretory vs. CNS prions. Understanding the mechanisms that enable efficient
prion dissemination and shedding will help elucidate how horizontally
transmissible prions evolve and succeed, and is the basis of this proposal.
Understanding how infectious misfolded proteins (prions) are generated,
trafficked, shed, and transmitted will aid in preventing, treating, and
managing the risks associated with these agents and the diseases they cause.
Public Health Relevance Chronic
wasting disease (CWD) of deer and elk is an emergent highly transmissible prion
disease now recognized throughout the USA as well as in Canada and Korea. We
have shown that infected deer harbor and shed high levels of infectious prions
in saliva, blood, urine, and feces thereby leading to transmission by direct
contact and environmental contamination. In that our studies have also shown
that CWD can infect some non-cervid species, the potential risk CWD may represents
to domestic animal species and humans remains unknown. The increasing parallels
in the development of major human neurodegenerative conditions, such as
Alzheimer's and Parkinson's diseases, and prion diseases add relevance to CWD
as a model of a transmissible protein misfolding disease. Understanding how
infectious misfolded proteins (prions) are generated and transmitted will aid
in interrupting, treating, and managing the risks associated with these agents
and the diseases they cause.
Funding Agency Agency National
Institute of Health (NIH)
Institute National Institute of
Neurological Disorders and Stroke (NINDS)
Type Research Project (R01)
Project # 4R01NS061902-07
Application # 9010980
Study Section Cellular and
Molecular Biology of Neurodegeneration Study Section (CMND)
Program Officer Wong, May Project
Start 2009-09-30
Project End 2018-02-28
Budget Start 2016-03-01
Budget End 2017-02-28
Support Year 7
Fiscal Year 2016
Total Cost $409,868
Indirect Cost $134,234 Institution
Name Colorado State University-Fort Collins
Department
Microbiology/Immun/Virology
Type Schools of Veterinary Medicine
DUNS # 785979618 City Fort Collins
State CO
Country United States
Zip Code 80523
LOOKING FOR CWD IN HUMANS AS nvCJD
or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$
*** These results would seem to
suggest that CWD does indeed have zoonotic potential, at least as judged by the
compatibility of CWD prions and their human PrPC target. Furthermore,
extrapolation from this simple in vitro assay suggests that if zoonotic CWD
occurred, it would most likely effect those of the PRNP codon 129-MM genotype
and that the PrPres type would be similar to that found in the most common
subtype of sCJD (MM1).***
PRION 2015 CONFERENCE FT. COLLINS
CWD RISK FACTORS TO HUMANS
*** LATE-BREAKING ABSTRACTS PRION
2015 CONFERENCE ***
O18
Zoonotic Potential of CWD Prions
Liuting Qing1, Ignazio Cali1,2, Jue
Yuan1, Shenghai Huang3, Diane Kofskey1, Pierluigi Gambetti1, Wenquan Zou1,
Qingzhong Kong1 1Case Western Reserve University, Cleveland, Ohio, USA, 2Second
University of Naples, Naples, Italy, 3Encore Health Resources, Houston, Texas,
USA
*** These results indicate that the
CWD prion has the potential to infect human CNS and peripheral lymphoid tissues
and that there might be asymptomatic human carriers of CWD infection.
==================
***These results indicate that the
CWD prion has the potential to infect human CNS and peripheral lymphoid tissues
and that there might be asymptomatic human carriers of CWD infection.***
==================
P.105: RT-QuIC models trans-species
prion transmission
Kristen Davenport, Davin Henderson,
Candace Mathiason, and Edward Hoover Prion Research Center; Colorado State
University; Fort Collins, CO USA
Conversely, FSE maintained
sufficient BSE characteristics to more efficiently convert bovine rPrP than
feline rPrP. Additionally, human rPrP was competent for conversion by CWD and
fCWD.
***This insinuates that, at the
level of protein:protein interactions, the barrier preventing transmission of
CWD to humans is less robust than previously estimated.
================
***This insinuates that, at the
level of protein:protein interactions, the barrier preventing transmission of
CWD to humans is less robust than previously estimated.***
================
*** PRICE OF CWD TSE PRION POKER
GOES UP 2014 ***
Transmissible Spongiform
Encephalopathy TSE PRION update January 2, 2014
*** chronic wasting disease, there
was no absolute barrier to conversion of the human prion protein.
*** Furthermore, the form of human
PrPres produced in this in vitro assay when seeded with CWD, resembles that
found in the most common human prion disease, namely sCJD of the MM1 subtype.
*** These results would seem to
suggest that CWD does indeed have zoonotic potential, at least as judged by the
compatibility of CWD prions and their human PrPC target. Furthermore,
extrapolation from this simple in vitro assay suggests that if zoonotic CWD
occurred, it would most likely effect those of the PRNP codon 129-MM genotype
and that the PrPres type would be similar to that found in the most common
subtype of sCJD (MM1).***
*** The potential impact of prion
diseases on human health was greatly magnified by the recognition that
interspecies transfer of BSE to humans by beef ingestion resulted in vCJD.
While changes in animal feed constituents and slaughter practices appear to
have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in
the U.S. might also cross the species barrier. Thus, consuming venison could be
a source of human prion disease. Whether BSE and CWD represent interspecies
scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the
possibility of transmission of prion disease through other food animals cannot
be ruled out. There is evidence that vCJD can be transmitted through blood
transfusion. There is likely a pool of unknown size of asymptomatic individuals
infected with vCJD, and there may be asymptomatic individuals infected with the
CWD equivalent. These circumstances represent a potential threat to blood,
blood products, and plasma supplies.
***********CJD REPORT 1994
increased risk for consumption of veal and venison and lamb***********
CREUTZFELDT JAKOB DISEASE
SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994
Consumption of venison and veal was
much less widespread among both cases and controls. For both of these meats
there was evidence of a trend with increasing frequency of consumption being
associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss)
These associations were largely
unchanged when attention was restricted to pairs with data obtained from
relatives. ...
Table 9 presents the results of an
analysis of these data.
There is STRONG evidence of an
association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).
Individuals reported to eat veal on
average at least once a year appear to be at 13 TIMES THE RISK of individuals
who have never eaten veal.
There is, however, a very wide
confidence interval around this estimate. There is no strong evidence that
eating veal less than once per year is associated with increased risk of CJD (p
= 0.51).
The association between venison
eating and risk of CJD shows similar pattern, with regular venison eating
associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).
There is some evidence that risk of
CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).
The evidence for such an
association between beef eating and CJD is weaker (p = 0.14). When only
controls for whom a relative was interviewed are included, this evidence
becomes a little STRONGER (p = 0.08).
snip...
It was found that when veal was
included in the model with another exposure, the association between veal and
CJD remained statistically significant (p = < 0.05 for all exposures), while
the other exposures ceased to be statistically significant (p = > 0.05).
snip...
In conclusion, an analysis of
dietary histories revealed statistical associations between various
meats/animal products and INCREASED RISK OF CJD. When some account was taken of
possible confounding, the association between VEAL EATING AND RISK OF CJD
EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...
snip...
In the study in the USA, a range of
foodstuffs were associated with an increased risk of CJD, including liver
consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK
OF CJD. By comparing the data from 3 studies in relation to this particular
dietary factor, the risk of liver consumption became non-significant with an
odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS
UNIVERSITY, ROTTERDAM). (???...TSS)
snip...see full report ;
CJD9/10022
October 1994
Mr R.N. Elmhirst Chairman British
Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ
Dear Mr Elmhirst,
CREUTZFELDT-JAKOB DISEASE (CJD)
SURVEILLANCE UNIT REPORT
Thank you for your recent letter
concerning the publication of the third annual report from the CJD Surveillance
Unit. I am sorry that you are dissatisfied with the way in which this report
was published.
The Surveillance Unit is a completely
independant outside body and the Department of Health is committed to
publishing their reports as soon as they become available. In the circumstances
it is not the practice to circulate the report for comment since the findings
of the report would not be amended. In future we can ensure that the British
Deer Farmers Association receives a copy of the report in advance of
publication.
The Chief Medical Officer has
undertaken to keep the public fully informed of the results of any research in
respect of CJD. This report was entirely the work of the unit and was produced
completely independantly of the the Department.
The statistical results reqarding
the consumption of venison was put into perspective in the body of the report
and was not mentioned at all in the press release. Media attention regarding
this report was low key but gave a realistic presentation of the statistical
findings of the Unit. This approach to publication was successful in that
consumption of venison was highlighted only once by the media ie. in the News
at one television proqramme.
I believe that a further statement
about the report, or indeed statistical links between CJD and consumption of
venison, would increase, and quite possibly give damaging credence, to the
whole issue. From the low key media reports of which I am aware it seems
unlikely that venison consumption will suffer adversely, if at all.
http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
Monday, May 02, 2016
*** Zoonotic Potential of CWD
Prions: An Update Prion 2016 Tokyo ***
*** PRION 2014 CONFERENCE CHRONIC
WASTING DISEASE CWD
http://chronic-wasting-disease.blogspot.com/
Monday, May 02, 2016
*** Zoonotic Potential of CWD
Prions: An Update Prion 2016 Tokyo ***
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
*** 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.
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.
==============
SCRAPIE WS-01: Prion diseases in
animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN:
1933-6896 printl 1933-690X online
***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.
please see file attachment for full
submission and recent science and my deep concerns on the TSE Prion disease...
No documents available. AttachmentsView All (1) scrapie-usa-blogspot-com View
Attachment:
Saturday, July 16, 2016
Importation of Sheep, Goats, and
Certain Other Ruminants [Docket No. APHIS-2009-0095]RIN 0579-AD10
WITH great disgust and concern, I
report to you that the OIE, USDA, APHIS, are working to further legalize the
trading of Transmissible Spongiform Encephalopathy TSE Pion disease around the
globe.
THIS is absolutely insane. it’s
USDA INC.
2016 Comment from Terry Singeltary
Sr.
The is a Comment on the Food and
Drug Administration (FDA) Notice: 158 Guidance for Industry Use of Material
from Deer and Elk in Animal Feed
DOCKET-- 03D-0186 -- FDA Issues
Draft Guidance on Use of Material From Deer and Elk in Animal Feed;
Availability Date: Fri, 16 May 2003 11:47:37 0500 EMC 1 Terry S. Singeltary Sr.
Vol #: 1
PLEASE SEE FULL TEXT SUBMISSION ;
*** 2001 Terry S. Singeltary Sr.
comment submission ***
Wednesday, November 09, 2016
Maine Medical Center postpones
elective surgeries over suspected case of prion disease
Using in vitro prion replication
for high sensitive detection of prions and prionlike proteins and for
understanding mechanisms of transmission.
Claudio Soto
Mitchell Center for Alzheimer's
diseases and related Brain disorders, Department of Neurology, University of
Texas Medical School at Houston.
Prion and prion-like proteins are
misfolded protein aggregates with the ability to selfpropagate to spread
disease between cells, organs and in some cases across individuals. I n T r a n
s m i s s i b l e s p o n g i f o r m encephalopathies (TSEs), prions are
mostly composed by a misfolded form of the prion protein (PrPSc), which
propagates by transmitting its misfolding to the normal prion protein (PrPC).
The availability of a procedure to replicate prions in the laboratory may be
important to study the mechanism of prion and prion-like spreading and to
develop high sensitive detection of small quantities of misfolded proteins in
biological fluids, tissues and environmental samples. Protein Misfolding Cyclic
Amplification (PMCA) is a simple, fast and efficient methodology to mimic prion
replication in the test tube. PMCA is a platform technology that may enable
amplification of any prion-like misfolded protein aggregating through a
seeding/nucleation process. In TSEs, PMCA is able to detect the equivalent of
one single molecule of infectious PrPSc and propagate prions that maintain high
infectivity, strain properties and species specificity. Using PMCA we have been
able to detect PrPSc in blood and urine of experimentally infected animals and
humans affected by vCJD with high sensitivity and specificity. Recently, we
have expanded the principles of PMCA to amplify amyloid-beta (Aß) and
alphasynuclein (a-syn) aggregates implicated in Alzheimer's and Parkinson's diseases,
respectively. Experiments are ongoing to study the utility of this technology
to detect Aß and a-syn aggregates in samples of CSF and blood from patients
affected by these diseases.
=========================
***Recently, we have been using
PMCA to study the role of environmental prion contamination on the horizontal
spreading of TSEs. These experiments have focused on the study of the
interaction of prions with plants and environmentally relevant surfaces. Our
results show that plants (both leaves and roots) bind tightly to prions present
in brain extracts and excreta (urine and feces) and retain even small
quantities of PrPSc for long periods of time. Strikingly, ingestion of
prioncontaminated leaves and roots produced disease with a 100% attack rate and
an incubation period not substantially longer than feeding animals directly
with scrapie brain homogenate. Furthermore, plants can uptake prions from
contaminated soil and transport them to different parts of the plant tissue
(stem and leaves). Similarly, prions bind tightly to a variety of
environmentally relevant surfaces, including stones, wood, metals, plastic,
glass, cement, etc. Prion contaminated surfaces efficiently transmit prion
disease when these materials were directly injected into the brain of animals
and strikingly when the contaminated surfaces were just placed in the animal
cage. These findings demonstrate that environmental materials can efficiently
bind infectious prions and act as carriers of infectivity, suggesting that they
may play an important role in the horizontal transmission of the disease.
========================
Since its invention 13 years ago,
PMCA has helped to answer fundamental questions of prion propagation and has
broad applications in research areas including the food industry, blood bank
safety and human and veterinary disease diagnosis.
see ;
with CWD TSE Prions, I am not sure
there is any absolute yet, other than what we know with transmission studies,
and we know tse prion kill, and tse prion are bad. science shows to date, that
indeed soil, dirt, some better than others, can act as a carrier. same with
objects, farm furniture. take it with how ever many grains of salt you wish, or
not. if load factor plays a role in the end formula, then everything should be
on the table, in my opinion. see ; ***Recently, we have been using PMCA to
study the role of environmental prion contamination on the horizontal spreading
of TSEs. These experiments have focused on the study of the interaction of
prions with plants and environmentally relevant surfaces. Our results show that
plants (both leaves and roots) bind tightly to prions present in brain extracts
and excreta (urine and feces) and retain even small quantities of PrPSc for
long periods of time. Strikingly, ingestion of prioncontaminated leaves and
roots produced disease with a 100% attack rate and an incubation period not substantially
longer than feeding animals directly with scrapie brain homogenate.
Furthermore, plants can uptake prions from contaminated soil and transport them
to different parts of the plant tissue (stem and leaves). Similarly, prions
bind tightly to a variety of environmentally relevant surfaces, including
stones, wood, metals, plastic, glass, cement, etc. Prion contaminated surfaces
efficiently transmit prion disease when these materials were directly injected
into the brain of animals and strikingly when the contaminated surfaces were
just placed in the animal cage. These findings demonstrate that environmental
materials can efficiently bind infectious prions and act as carriers of
infectivity, suggesting that they may play an important role in the horizontal
transmission of the disease.
Since its invention 13 years ago,
PMCA has helped to answer fundamental questions of prion propagation and has
broad applications in research areas including the food industry, blood bank
safety and human and veterinary disease diagnosis.
see ;
Oral Transmissibility of Prion
Disease Is Enhanced by Binding to Soil Particles
Author Summary
Transmissible spongiform
encephalopathies (TSEs) are a group of incurable neurological diseases likely caused
by a misfolded form of the prion protein. TSEs include scrapie in sheep, bovine
spongiform encephalopathy (‘‘mad cow’’ disease) in cattle, chronic wasting
disease in deer and elk, and Creutzfeldt-Jakob disease in humans. Scrapie and
chronic wasting disease are unique among TSEs because they can be transmitted
between animals, and the disease agents appear to persist in environments
previously inhabited by infected animals. Soil has been hypothesized to act as
a reservoir of infectivity and to bind the infectious agent. In the current
study, we orally dosed experimental animals with a common clay mineral,
montmorillonite, or whole soils laden with infectious prions, and compared the
transmissibility to unbound agent. We found that prions bound to montmorillonite
and whole soils remained orally infectious, and, in most cases, increased the
oral transmission of disease compared to the unbound agent. The results
presented in this study suggest that soil may contribute to environmental
spread of TSEs by increasing the transmissibility of small amounts of
infectious agent in the environment.
https://www.aphis.usda.gov/emergency_response/downloads/tools/johnson
et al prions in soil.pdf
tse prion soil
Wednesday, December 16, 2015
Objects in contact with classical
scrapie sheep act as a reservoir for scrapie transmission
The sources of dust borne prions
are unknown but it seems reasonable to assume that faecal, urine, skin,
parturient material and saliva-derived prions may contribute to this mobile
environmental reservoir of infectivity. This work highlights a possible
transmission route for scrapie within the farm environment, and this is likely
to be paralleled in CWD which shows strong similarities with scrapie in terms
of prion dissemination and disease transmission. The data indicate that the
presence of scrapie prions in dust is likely to make the control of these
diseases a considerable challenge.
>>>Particle-associated
PrPTSE molecules may migrate from locations of deposition via transport
processes affecting soil particles, including entrainment in and movement with
air and overland flow. <<<
Fate of Prions in Soil: A Review
Christen B. Smith, Clarissa J.
Booth, and Joel A. Pedersen*
Several reports have shown that
prions can persist in soil for several years. Significant interest remains in
developing methods that could be applied to degrade PrPTSE in naturally
contaminated soils. Preliminary research suggests that serine proteases and the
microbial consortia in stimulated soils and compost may partially degrade
PrPTSE. Transition metal oxides in soil (viz. manganese oxide) may also mediate
prion inactivation. Overall, the effect of prion attachment to soil particles
on its persistence in the environment is not well understood, and additional
study is needed to determine its implications on the environmental transmission
of scrapie and CWD.
P.161: Prion soil binding may
explain efficient horizontal CWD transmission
Conclusion. Silty clay loam
exhibits highly efficient prion binding, inferring a durable environmental
reservoir, and an efficient mechanism for indirect horizontal CWD transmission.
>>>Another alternative
would be an absolute prohibition on the movement of deer within the state for
any purpose. While this alternative would significantly reduce the potential
spread of CWD, it would also have the simultaneous effect of preventing landowners
and land managers from implementing popular management strategies involving the
movement of deer, and would deprive deer breeders of the ability to engage in
the business of buying and selling breeder deer. Therefore, this alternative
was rejected because the department determined that it placed an avoidable
burden on the regulated community.<<<
Wednesday, December 16, 2015
Objects in contact with classical
scrapie sheep act as a reservoir for scrapie transmission
Objects in contact with classical
scrapie sheep act as a reservoir for scrapie transmission
Timm Konold1*, Stephen A. C.
Hawkins2, Lisa C. Thurston3, Ben C. Maddison4, Kevin C. Gough5, Anthony Duarte1
and Hugh A. Simmons1
1 Animal Sciences Unit, Animal and
Plant Health Agency Weybridge, Addlestone, UK, 2 Pathology Department, Animal
and Plant Health Agency Weybridge, Addlestone, UK, 3 Surveillance and
Laboratory Services, Animal and Plant Health Agency Penrith, Penrith, UK, 4
ADAS UK, School of Veterinary Medicine and Science, University of Nottingham,
Sutton Bonington, UK, 5 School of Veterinary Medicine and Science, University
of Nottingham, Sutton Bonington, UK Classical scrapie is an environmentally
transmissible prion disease of sheep and goats. Prions can persist and remain
potentially infectious in the environment for many years and thus pose a risk
of infecting animals after re-stocking. In vitro studies using serial protein
misfolding cyclic amplification (sPMCA) have suggested that objects on a
scrapie affected sheep farm could contribute to disease transmission. This in
vivo study aimed to determine the role of field furniture (water troughs,
feeding troughs, fencing, and other objects that sheep may rub against) used by
a scrapie-infected sheep flock as a vector for disease transmission to
scrapie-free lambs with the prion protein genotype VRQ/VRQ, which is associated
with high susceptibility to classical scrapie. When the field furniture was
placed in clean accommodation, sheep became infected when exposed to either a
water trough (four out of five) or to objects used for rubbing (four out of
seven). This field furniture had been used by the scrapie-infected flock 8
weeks earlier and had previously been shown to harbor scrapie prions by sPMCA.
Sheep also became infected (20 out of 23) through exposure to contaminated
field furniture placed within pasture not used by scrapie-infected sheep for 40
months, even though swabs from this furniture tested negative by PMCA. This
infection rate decreased (1 out of 12) on the same paddock after replacement
with clean field furniture. Twelve grazing sheep exposed to field furniture not
in contact with scrapie-infected sheep for 18 months remained scrapie free. The
findings of this study highlight the role of field furniture used by
scrapie-infected sheep to act as a reservoir for disease re-introduction
although infectivity declines considerably if the field furniture has not been
in contact with scrapie-infected sheep for several months. PMCA may not be as
sensitive as VRQ/VRQ sheep to test for environmental contamination.
snip...
Discussion
Classical scrapie is an
environmentally transmissible disease because it has been reported in naïve,
supposedly previously unexposed sheep placed in pastures formerly occupied by
scrapie-infected sheep (4, 19, 20). Although the vector for disease
transmission is not known, soil is likely to be an important reservoir for
prions (2) where – based on studies in rodents – prions can adhere to minerals
as a biologically active form (21) and remain infectious for more than 2 years
(22). Similarly, chronic wasting disease (CWD) has re-occurred in mule deer
housed in paddocks used by infected deer 2 years earlier, which was assumed to
be through foraging and soil consumption (23).
Our study suggested that the risk of
acquiring scrapie infection was greater through exposure to contaminated
wooden, plastic, and metal surfaces via water or food troughs, fencing, and
hurdles than through grazing. Drinking from a water trough used by the scrapie
flock was sufficient to cause infection in sheep in a clean building. Exposure
to fences and other objects used for rubbing also led to infection, which
supported the hypothesis that skin may be a vector for disease transmission
(9). The risk of these objects to cause infection was further demonstrated when
87% of 23 sheep presented with PrPSc in lymphoid tissue after grazing on one of
the paddocks, which contained metal hurdles, a metal lamb creep and a water
trough in contact with the scrapie flock up to 8 weeks earlier, whereas no
infection had been demonstrated previously in sheep grazing on this paddock,
when equipped with new fencing and field furniture. When the contaminated
furniture and fencing were removed, the infection rate dropped significantly to
8% of 12 sheep, with soil of the paddock as the most likely source of infection
caused by shedding of prions from the scrapie-infected sheep in this paddock up
to a week earlier.
This study also indicated that the
level of contamination of field furniture sufficient to cause infection was
dependent on two factors: stage of incubation period and time of last use by
scrapie-infected sheep. Drinking from a water trough that had been used by
scrapie sheep in the predominantly pre-clinical phase did not appear to cause
infection, whereas infection was shown in sheep drinking from the water trough
used by scrapie sheep in the later stage of the disease. It is possible that
contamination occurred through shedding of prions in saliva, which may have
contaminated the surface of the water trough and subsequently the water when it
was refilled. Contamination appeared to be sufficient to cause infection only
if the trough was in contact with sheep that included clinical cases. Indeed,
there is an increased risk of bodily fluid infectivity with disease progression
in scrapie (24) and CWD (25) based on PrPSc detection by sPMCA. Although
ultraviolet light and heat under natural conditions do not inactivate prions
(26), furniture in contact with the scrapie flock, which was assumed to be
sufficiently contaminated to cause infection, did not act as vector for disease
if not used for 18 months, which suggest that the weathering process alone was
sufficient to inactivate prions.
PrPSc detection by sPMCA is
increasingly used as a surrogate for infectivity measurements by bioassay in
sheep or mice. In this reported study, however, the levels of PrPSc present in
the environment were below the limit of detection of the sPMCA method, yet were
still sufficient to cause infection of in-contact animals. In the present
study, the outdoor objects were removed from the infected flock 8 weeks prior
to sampling and were positive by sPMCA at very low levels (2 out of 37
reactions). As this sPMCA assay also yielded 2 positive reactions out of 139 in
samples from the scrapie-free farm, the sPMCA assay could not detect PrPSc on
any of the objects above the background of the assay. False positive reactions
with sPMCA at a low frequency associated with de novo formation of infectious
prions have been reported (27, 28). This is in contrast to our previous study
where we demonstrated that outdoor objects that had been in contact with the
scrapie-infected flock up to 20 days prior to sampling harbored PrPSc that was
detectable by sPMCA analysis [4 out of 15 reactions (12)] and was significantly
more positive by the assay compared to analogous samples from the scrapie-free
farm. This discrepancy could be due to the use of a different sPMCA substrate
between the studies that may alter the efficiency of amplification of the environmental
PrPSc. In addition, the present study had a longer timeframe between the
objects being in contact with the infected flock and sampling, which may affect
the levels of extractable PrPSc. Alternatively, there may be potentially patchy
contamination of this furniture with PrPSc, which may have been missed by
swabbing. The failure of sPMCA to detect CWD-associated PrP in saliva from
clinically affected deer despite confirmation of infectivity in
saliva-inoculated transgenic mice was associated with as yet unidentified
inhibitors in saliva (29), and it is possible that the sensitivity of sPMCA is
affected by other substances in the tested material. In addition, sampling of
amplifiable PrPSc and subsequent detection by sPMCA may be more difficult from
furniture exposed to weather, which is supported by the observation that PrPSc
was detected by sPMCA more frequently in indoor than outdoor furniture (12). A
recent experimental study has demonstrated that repeated cycles of drying and
wetting of prion-contaminated soil, equivalent to what is expected under
natural weathering conditions, could reduce PMCA amplification efficiency and
extend the incubation period in hamsters inoculated with soil samples (30).
This seems to apply also to this study even though the reduction in infectivity
was more dramatic in the sPMCA assays than in the sheep model. Sheep were not
kept until clinical end-point, which would have enabled us to compare
incubation periods, but the lack of infection in sheep exposed to furniture that
had not been in contact with scrapie sheep for a longer time period supports
the hypothesis that prion degradation and subsequent loss of infectivity occurs
even under natural conditions.
In conclusion, the results in the
current study indicate that removal of furniture that had been in contact with
scrapie-infected animals should be recommended, particularly since cleaning and
decontamination may not effectively remove scrapie infectivity (31), even
though infectivity declines considerably if the pasture and the field furniture
have not been in contact with scrapie-infected sheep for several months. As
sPMCA failed to detect PrPSc in furniture that was subjected to weathering,
even though exposure led to infection in sheep, this method may not always be reliable
in predicting the risk of scrapie infection through environmental
contamination. These results suggest that the VRQ/VRQ sheep model may be more
sensitive than sPMCA for the detection of environmentally associated scrapie,
and suggest that extremely low levels of scrapie contamination are able to
cause infection in susceptible sheep genotypes.
Keywords: classical scrapie, prion,
transmissible spongiform encephalopathy, sheep, field furniture, reservoir,
serial protein misfolding cyclic amplification
Wednesday, December 16, 2015
*** Objects in contact with
classical scrapie sheep act as a reservoir for scrapie transmission ***
*** Infectious agent of sheep
scrapie may persist in the environment for at least 16 years ***
Gudmundur Georgsson1, Sigurdur
Sigurdarson2 and Paul Brown3
>>>Another alternative
would be an absolute prohibition on the movement of deer within the state for
any purpose. While this alternative would significantly reduce the potential
spread of CWD, it would also have the simultaneous effect of preventing
landowners and land managers from implementing popular management strategies
involving the movement of deer, and would deprive deer breeders of the ability
to engage in the business of buying and selling breeder deer. Therefore, this
alternative was rejected because the department determined that it placed an
avoidable burden on the regulated community.<<< Circulation of prions
within dust on a scrapie affected farm Kevin C Gough1, Claire A Baker2, Hugh A
Simmons3, Steve A Hawkins3 and Ben C Maddison2*
Abstract
Prion diseases are fatal
neurological disorders that affect humans and animals. Scrapie of sheep/goats
and Chronic Wasting Disease (CWD) of deer/elk are contagious prion diseases
where environmental reservoirs have a direct link to the transmission of
disease. Using protein misfolding cyclic amplification we demonstrate that
scrapie PrPSc can be detected within circulating dusts that are present on a
farm that is naturally contaminated with sheep scrapie. The presence of
infectious scrapie within airborne dusts may represent a possible route of
infection and illustrates the difficulties that may be associated with the
effective decontamination of such scrapie affected premises.
snip...
Discussion
We present biochemical data
illustrating the airborne movement of scrapie containing material within a
contaminated farm environment. We were able to detect scrapie PrPSc within
extracts from dusts collected over a 70 day period, in the absence of any sheep
activity. We were also able to detect scrapie PrPSc within dusts collected
within pasture at 30 m but not at 60 m distance away from the scrapie
contaminated buildings, suggesting that the chance of contamination of pasture
by scrapie contaminated dusts decreases with distance from contaminated farm
buildings. PrPSc amplification by sPMCA has been shown to correlate with
infectivity and amplified products have been shown to be infectious [14,15].
These experiments illustrate the potential for low dose scrapie infectivity to
be present within such samples. We estimate low ng levels of scrapie positive
brain equivalent were deposited per m2 over 70 days, in a barn previously
occupied by sheep affected with scrapie. This movement of dusts and the
accumulation of low levels of scrapie infectivity within this environment may
in part explain previous observations where despite stringent pen
decontamination regimens healthy lambs still became scrapie infected after
apparent exposure from their environment alone [16]. The presence of sPMCA
seeding activity and by inference, infectious prions within dusts, and their
potential for airborne dissemination is highly novel and may have implications
for the spread of scrapie within infected premises. The low level circulation
and accumulation of scrapie prion containing dust material within the farm
environment will likely impede the efficient decontamination of such scrapie
contaminated buildings unless all possible reservoirs of dust are removed.
Scrapie containing dusts could possibly infect animals during feeding and
drinking, and respiratory and conjunctival routes may also be involved. It has
been demonstrated that scrapie can be efficiently transmitted via the nasal
route in sheep [17], as is also the case for CWD in both murine models and in
white tailed deer [18-20].
The sources of dust borne prions
are unknown but it seems reasonable to assume that faecal, urine, skin,
parturient material and saliva-derived prions may contribute to this mobile
environmental reservoir of infectivity. This work highlights a possible transmission
route for scrapie within the farm environment, and this is likely to be
paralleled in CWD which shows strong similarities with scrapie in terms of
prion dissemination and disease transmission. The data indicate that the
presence of scrapie prions in dust is likely to make the control of these
diseases a considerable challenge.
Saturday, December 12, 2015
NOTICE: Environmental Impact
Statement on Large Livestock Carcasses TSE Prion REPORT December 14, 2015
Friday, August 14, 2015
Carcass Management During a Mass
Animal Health Emergency Draft Programmatic Environmental Impact Statement—August
2015
Wednesday, September 7, 2016
An assessment of the long-term
persistence of prion infectivity in aquatic environments
SATURDAY, OCTOBER 08, 2016
Modeled Impacts of Chronic Wasting
Disease on White-Tailed Deer in a Semi-Arid Environment
SUNDAY, OCTOBER 02, 2016
What is the risk of a cervid TSE
being introduced from Norway into Great Britain? Qualitative Risk Assessment
September 2016
Title: Pathological features of
chronic wasting disease in reindeer and demonstration of horizontal
transmission Author
item Moore, Sarah item Kunkle,
Robert item West greenlee, Mary item Nicholson, Eric item Richt, Juergen item
Hamir, Amirali item Waters, Wade item Greenlee, Justin
Submitted to: Emerging Infectious
Diseases Publication Type: Peer reviewed journal Publication Acceptance Date:
8/29/2016 Publication Date: N/A Citation:
Interpretive Summary: Chronic
wasting disease (CWD) is a fatal neurodegenerative disease that occurs in
farmed and wild cervids (deer and elk) of North America and was recently
diagnosed in a single free-ranging reindeer (Rangifer tarandus tarandus) in
Norway. CWD is a transmissible spongiform encephalopathy (TSE) that is caused
by infectious proteins called prions that are resistant to various methods of
decontamination and environmental degradation. Little is known about the
susceptibility of or potential for transmission amongst reindeer. In this
experiment, we tested the susceptibility of reindeer to CWD from various
sources (elk, mule deer, or white-tailed deer) after intracranial inoculation
and tested the potential for infected reindeer to transmit to non-inoculated
animals by co-housing or housing in adjacent pens. Reindeer were susceptible to
CWD from elk, mule deer, or white-tailed deer sources after experimental
inoculation. Most importantly, non-inoculated reindeer that were co-housed with
infected reindeer or housed in pens adjacent to infected reindeer but without
the potential for nose-to-nose contact also developed evidence of CWD
infection. This is a major new finding that may have a great impact on the
recently diagnosed case of CWD in the only remaining free-ranging reindeer
population in Europe as our findings imply that horizontal transmission to
other reindeer within that herd has already occurred. Further, this information
will help regulatory and wildlife officials developing plans to reduce or
eliminate CWD and cervid farmers that want to ensure that their herd remains
CWD-free, but were previously unsure of the potential for reindeer to transmit
CWD.
Technical Abstract: Chronic wasting
disease (CWD) is a naturally-occurring, fatal prion disease of cervids. Reindeer
(Rangifer tarandus tarandus) are susceptible to CWD following oral challenge,
and CWD was recently reported in a free-ranging reindeer of Norway. Potential
contact between CWD-affected cervids and Rangifer species that are free-ranging
or co-housed on farms presents a potential risk of CWD transmission. The aims
of this study were to 1) investigate the transmission of CWD from white-tailed
deer (Odocoileus virginianus; CWDwtd), mule deer (Odocoileus hemionus; CWDmd),
or elk (Cervus elaphus nelsoni; CWDelk) to reindeer via the intracranial route,
and 2) to assess for direct and indirect horizontal transmission to
non-inoculated sentinels. Three groups of 5 reindeer fawns were challenged
intracranially with CWDwtd, CWDmd, or CWDelk. Two years after challenge of
inoculated reindeer, non-inoculated negative control reindeer were introduced
into the same pen as the CWDwtd inoculated reindeer (direct contact; n=4) or
into a pen adjacent to the CWDmd inoculated reindeer (indirect contact; n=2).
Experimentally inoculated reindeer were allowed to develop clinical disease. At
death/euthanasia a complete necropsy examination was performed, including
immunohistochemical testing of tissues for disease-associated CWD prion protein
(PrPcwd). Intracranially challenged reindeer developed clinical disease from 21
months post-inoculation (months PI). PrPcwd was detected in 5 out of 6 sentinel
reindeer although only 2 out of 6 developed clinical disease during the study
period (< 57 months PI). We have shown that reindeer are susceptible to CWD
from various cervid sources and can transmit CWD to naïve reindeer both
directly and indirectly.
Monday, September 05, 2016
*** Pathological features of
chronic wasting disease in reindeer and demonstration of horizontal transmission
Major Findings for Norway ***
Monday, September 05, 2016
Pathological features of chronic
wasting disease in reindeer and demonstration of horizontal transmission Major
Findings for Norway
Thursday, September 22, 2016
NORWAY DETECTS 5TH CASE OF CHRONIC
WASTING DISEASE CWD TSE PRION Skrantesjuke
Monday, August 29, 2016
*** NWHC USGS CHRONIC WASTING
DISEASE CWD TSE PRION UPDATE
Thursday, August 18, 2016
*** PROCEEDINGS ONE HUNDRED AND Nineteenth
ANNUAL MEETING of the USAHA BSE, CWD, SCRAPIE, PORCINE TSE PRION October 22 28,
2015 ***
Sunday, August 28, 2016
CONFIDENTIAL
Transmissible Spongiform
Encephalopathy TSE Prion and how Politics and Greed by the Industry spread
madcow type diseases from species to species and around the globe
TSE PRIONS AKA MAD COW TYPE
DISEASE, LIONS AND TIGERS AND BEARS, OH MY!
*** Infectious agent of sheep
scrapie may persist in the environment for at least 16 years ***
Gudmundur Georgsson1, Sigurdur
Sigurdarson2 and Paul Brown3
Saturday, May 28, 2016
*** Infection and detection of
PrPCWD in soil from CWD infected farm in Korea Prion 2016 Tokyo ***
*** COMMERCIAL IN CONFIDENCE ***
SPREADING OF UNPROCESSED BLOOD ON
LAND
SCRAPIE SEMEN COVER-UP
snip...see full text ;
*** How Did CWD Get Way Down In
Medina County, Texas?
DISCUSSION Observations of natural
outbreaks of scrapie indicated that the disease spread from flock to flock by
the movement of infected, but apparently normal, sheep which were incubating
the disease.
There was no evidence that the
disease spread to adjacent flocks in the absent of such movements or that
vectors or other host species were involved in the spread of scrapie to sheep
or goats; however, these possibilities should be kept open...
Tuesday, June 07, 2016
*** Comparison of two US sheep
scrapie isolates supports identification as separate strains ***
Research Project: TRANSMISSION,
DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
The Commission received four
comments regarding adoption of the new rule, but there is no change to the rule
in response to the comments.
Two of the commenters told us to
"trust experts like Dr. Dan McBride and your advisory committee that was
already prepared for this issue. We must at all cost protect the whitetail herd
in the dense areas of the Texas Hill Country where any outbreak could lead to
panic and economic collapse of these communities where hunting dollars are
vital to these communities." The Commission appreciates the support of the
task force. Another comment indicated that "it will be tough to contain
free ranging deer since they range many miles during breeding season." The
Commission agrees that is a tough aspect to fully control the spread of the
disease, but the zones were sized in order to take that into account. Lastly, a
comment indicated that "in light of the Chronic Wasting Disease (CWD)
epidemic, which has jumped the border from New Mexico into Texas, Texas ought
to reevaluate its enthusiasm for land spreading sewage sludge bio solids on
farm land, grazing ranges, hay fields and dairy pastures where livestock and
deer ingest dirt and sludge with their fodder." The Commission has no
jurisdiction over that issue and that is not something addressed in this rule.
snip...more here ;
Saturday, May 16, 2015
Land Spreading of the TSE Prion
Disease, blood tank for feed, plants, vegetables, and sludge, stupid is as
stupid does
Thursday, September 24, 2015
TEXAS Hunters Asked to Submit
Samples for Chronic Wasting Disease CWD TSE Prion Testing
*** I cannot stress enough to all
of you, for the sake of your family and mine, before putting anything in the
freezer, have those deer tested for CWD. ...terry
New studies on the heat resistance
of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C
suggests an inorganic template of replication
The infectious agents responsible
for transmissible spongiform encephalopathy (TSE) are notoriously resistant to
most physical and chemical methods used for inactivating pathogens, including
heat. It has long been recognized, for example, that boiling is ineffective and
that higher temperatures are most efficient when combined with steam under
pressure (i.e., autoclaving). As a means of decontamination, dry heat is used
only at the extremely high temperatures achieved during incineration, usually
in excess of 600°C. It has been assumed, without proof, that incineration
totally inactivates the agents of TSE, whether of human or animal origin.
Prion Infected Meat-and-Bone Meal
Is Still Infectious after Biodiesel Production
Histochemical analysis of hamster
brains inoculated with the solid residue showed typical spongiform degeneration
and vacuolation. Re-inoculation of these brains into a new cohort of hamsters
led to onset of clinical scrapie symptoms within 75 days, suggesting that the
specific infectivity of the prion protein was not changed during the biodiesel
process. The biodiesel reaction cannot be considered a viable prion
decontamination method for MBM, although we observed increased survival time of
hamsters and reduced infectivity greater than 6 log orders in the solid MBM
residue. Furthermore, results from our study compare for the first time prion
detection by Western Blot versus an infectivity bioassay for analysis of
biodiesel reaction products. We could show that biochemical analysis alone is
insufficient for detection of prion infectivity after a biodiesel process.
*** Infectious agent of sheep
scrapie may persist in the environment for at least 16 years ***
Gudmundur Georgsson1, Sigurdur
Sigurdarson2 and Paul Brown3
Terry S. Singeltary Sr.
posted by Terry S. Singeltary Sr. at
1:54 PM
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