An assessment of the long-term persistence of prion infectivity in aquatic
environments
Alba Marín-Morenoa, Juan-Carlos Espinosaa, Natalia Fernández-Borgesa, Juan
Píquera, Rosina Gironesb, Olivier Andreolettic, Juan-María Torresa, ,
http://dx.doi.org/10.1016/j.envres.2016.08.031Get
rights and content
Highlights
• Prion infectivity resists long term incubations in aquatic environments.
• Infectivity persistence in wastewater is reduced when compared to PBS.
• In this study PrPRes fails as a marker for prion detection.
• Mice bioassay is the most powerful tool for assessing prion presence.
• Wastewater conventional treatment would not eliminate prion infectivity.
Abstract
The environment plays a key role in horizontal transmission of prion
diseases, since prions are extremely resistant to classical inactivation
procedures. In prior work, we observed the high stability of bovine spongiform
encephalopathy (BSE) infectivity when these prions were incubated in aqueous
media such as phosphate-buffered saline (PBS) or wastewater for nearly nine
months. As a continuation of this experiment, the same samples were maintained
in PBS or wastewater for five additional years and residual BSE infectivity was
assessed in bovine PrPC transgenic mice. Over this long time period (more than
six years), BSE infectivity was reduced by three and one orders of magnitude in
wastewater and PBS respectively. To rule out a possible agent specific effect,
sheep scrapie prions were subjected to the same experimental protocol, using
eight years as the experimental end-point. No significant reduction in scrapie
infectivity was observed over the first nine months of wastewater incubation
while PBS incubation for eight years only produced a two logarithmic unit
reduction in infectivity. By contrast, the dynamics of PrPRes persistence was
different, disappearing progressively over the first year. The long persistence
of prion infectivity observed in this study for two different agents provides
supporting evidence of the assumed high stability of these agents in aquatic
environments and that environmental processes or conventional wastewater
treatments with low retention times would have little impact on prion
infectivity. These results could have great repercussions in terms of risk
assessment and safety for animals and human populations.
Keywords Prion; Scrapie; BSE; Infectivity; Wastewater
*** The long persistence of prion infectivity observed in this study for
two different agents provides supporting evidence of the assumed high stability
of these agents in aquatic environments and that environmental processes or
conventional wastewater treatments with low retention times would have little
impact on prion infectivity.
*** These results could have great repercussions in terms of risk
assessment and safety for animals and human populations.
Detection of protease-resistant cervid prion protein in water from a
CWD-endemic area
The data presented here demonstrate that sPMCA can detect low levels of
PrPCWD in the environment, corroborate previous biological and experimental data
suggesting long term persistence of prions in the environment2,3 and imply that
PrPCWD accumulation over time may contribute to transmission of CWD in areas
where it has been endemic for decades. This work demonstrates the utility of
sPMCA to evaluate other environmental water sources for PrPCWD, including
smaller bodies of water such as vernal pools and wallows, where large numbers of
cervids congregate and into which prions from infected animals may be shed and
concentrated to infectious levels.
A Quantitative Assessment of the Amount of Prion Diverted to Category 1
Materials and Wastewater During Processing
Keywords:Abattoir;bovine spongiform encephalopathy;QRA;scrapie;TSE
In this article the development and parameterization of a quantitative
assessment is described that estimates the amount of TSE infectivity that is
present in a whole animal carcass (bovine spongiform encephalopathy [BSE] for
cattle and classical/atypical scrapie for sheep and lambs) and the amounts that
subsequently fall to the floor during processing at facilities that handle
specified risk material (SRM). BSE in cattle was found to contain the most oral
doses, with a mean of 9864 BO ID50s (310, 38840) in a whole carcass compared to
a mean of 1851 OO ID50s (600, 4070) and 614 OO ID50s (155, 1509) for a sheep
infected with classical and atypical scrapie, respectively. Lambs contained the
least infectivity with a mean of 251 OO ID50s (83, 548) for classical scrapie
and 1 OO ID50s (0.2, 2) for atypical scrapie. The highest amounts of infectivity
falling to the floor and entering the drains from slaughtering a whole carcass
at SRM facilities were found to be from cattle infected with BSE at rendering
and large incineration facilities with 7.4 BO ID50s (0.1, 29), intermediate
plants and small incinerators with a mean of 4.5 BO ID50s (0.1, 18), and
collection centers, 3.6 BO ID50s (0.1, 14). The lowest amounts entering drains
are from lambs infected with classical and atypical scrapie at intermediate
plants and atypical scrapie at collection centers with a mean of 3 × 10−7 OO
ID50s (2 × 10−8, 1 × 10−6) per carcass. The results of this model provide key
inputs for the model in the companion paper published here.
Monday, June 23, 2008
Persistence of Pathogenic Prion Protein during Simulated Wastewater
Treatment Processes
ASAP Environ. Sci. Technol., ASAP Article, 10.1021/es703186e Web Release
Date: June 10, 2008
Copyright © 2008 American Chemical Society
Persistence of Pathogenic Prion Protein during Simulated Wastewater
Treatment Processes
Glen T. Hinckley,†? Christopher J. Johnson,‡? Kurt H. Jacobson,? Christian
Bartholomay,§? Katherine D. McMahon,? Debbie McKenzie,? Judd M. Aiken,? and Joel
A. Pedersen*?#
Department of Civil and Environmental Engineering, Department of
Comparative Biosciences, and Department of Soil Science, University of
Wisconsin, Madison, Wisconsin 53706
Received for review December 19, 2007
Revised manuscript received April 4, 2008
Accepted April 9, 2008
Abstract:
Transmissible spongiform encephalopathies (TSEs, prion diseases) are a
class of fatal neurodegenerative diseases affecting a variety of mammalian
species including humans. A misfolded form of the prion protein (PrPTSE) is the
major, if not sole, component of the infectious agent. Prions are highly
resistant to degradation and to many disinfection procedures suggesting that, if
prions enter wastewater treatment systems through sewers and/or septic systems
(e.g., from slaughterhouses, necropsy laboratories, rural meat processors,
private game dressing) or through leachate from landfills that have received
TSE-contaminated material, prions could survive conventional wastewater
treatment. Here, we report the results of experiments examining the partitioning
and persistence of PrPTSE during simulated wastewater treatment processes
including activated and mesophilic anaerobic sludge digestion. Incubation with
activated sludge did not result in significant PrPTSE degradation. PrPTSE and
prion infectivity partitioned strongly to activated sludge solids and are
expected to enter biosolids treatment processes. A large fraction of PrPTSE
survived simulated mesophilic anaerobic sludge digestion. The small reduction in
recoverable PrPTSE after 20-d anaerobic sludge digestion appeared attributable
to a combination of declining extractability with time and microbial
degradation. Our results suggest that if prions were to enter municipal
wastewater treatment systems, most would partition to activated sludge solids,
survive mesophilic anaerobic digestion, and be present in treated biosolids.
snip...
We further emphasize that, to date, prions have not been reported in
wastewater influent, effluent or biosolids. Immunochemical methods (e.g.,
immunoblotting, enzyme-linked immunosorbent assays) lack the sensitivity needed
to detect prion protein in wastewater, biosolids and other environmental media.
Recent advances in prion detection (e.g., refs 40–42) may lead to methods that
are sufficiently sensitive to measure prions in environmental matrices.
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
Critical Reviews in Microbiology, 2013; 39(2): 139–151 © 2013 Informa
Healthcare USA, Inc. ISSN 1040-841X print/ISSN 1549-7828 online DOI:
10.3109/1040841X.2012.694410
REVIEW ARTICLE
Treatment alternatives of slaughterhouse wastes, and their effect on the
inactivation of different pathogens: A review
Ingrid H. Franke-Whittle and Heribert Insam
Institute of Microbiology, Leopold-Franzens University, Innsbruck,
Austria
There are many advantages of using AH for the inactivation of disease
agents in slaughterhouse wastes. These include the combination of a
sterilization and digestion 142 I. H. Franke-Whittle and H. Insam Critical
Reviews in Microbiology step into one operation, the reduction of waste volume
and weight by as much as 97%, the total destruction of pathogens including
prions such as BSE and the lower emission of odors or public nuisances.
snip...
BSE
BSE, also known as mad cow disease, is a relatively new disease that
primarily affects cattle. BSE can also cause a corresponding disease in
humans-Creutzfeldt-Jakob disease (Anon, 2003). There is still much controversy
regarding the causes of BSE and Creutzfeldt-Jakob disease, however the most
common belief is that the infectious agents are prions, an abnormal form of a
type of protein (Anon, 2003). However, the prion hypothesis has also been
challenged and an autoimmune response theory has been postulated (Ebringer et
al., 1997).
Currently, the public considers BSE to be the greatest concern to any
bovine-based product. The risk, however, of spreading BSE via composting of
catering wastes in the UK has been shown to be ‘remote’, because there are many
controls in place for keeping the disease from entering the food system, and
hence the food residuals stream in the UK today. In the slaughterhouse, TSE
management aims to prevent infected material from entering the food and feed
chains. According to EU legislation, animals suspected of TSE infection are
separated and safely disposed of. Prions such as BSE are more resistant to heat
than many viruses (Gale et al., 2004). In fact, BSE infected material remains
infected after cooking, rendering and long periods of incubation in the soil
(Anon, 2003). According to Rohwer (1984), less than a 0.5 log (70%) destruction
of scrapie agent was seen after 60 min at 60°C and 80°C. It can therefore be
assumed that a standard composting process whereby the temperature was
maintained at 60°C for 2 days would not reduce BSE infectivity.
146 I. H. Franke-Whittle and H. Insam Critical Reviews in
Microbiology
TSEs have also been reported to survive the operational temperatures at
which AD are conducted (Hinckley et al., 2008). Studies by Topper et al. (2006)
showed AD under both mesophilic and thermophilic conditions to be incapable of
reducing or eliminating BSE. In a study conducted by Brown et al. (2000),
infected brain material was heated to 600°C. Despite brains being totally ashed,
when reconstituted with saline to their original weights, ashed brain material
was able to transmit disease to 5 of 35 inoculated hamsters.
Apart from incineration (where temperatures of >850°C are reached;
Gwyther et al 2011), AH is the only effective method known for the destruction
of prion material (NABC 2004). A study carried out by the Institute of Animal
Health at the University of Edinburgh, investigated the ability of AH to destroy
BSE prions grown in the brains of mice. Infected mice heads were subjected to AH
for either 3 or 6 h, and after neutralization of the hydrolysates, aliquots were
injected into mice. Evidence of TSE was found in some mouse brains of mice
injected with hydrolysate taken from the 3 h digestion, but significantly, no
disease was found in the brains of mice injected with hydrolysate from the 6 h
digestion (NABC, 2004). Studies conducted by Murphy et al. (2009) also showed
scrapie to be inactivated by AH.
snip...
Conclusions
In the past, solid slaughterhouse wastes were most commonly treated by
rendering, the process providing slaughterhouses with an additional source of
income. However, because of the risk of TSEs, the economic value of such
products has been reduced significantly, and in fact, such products must in many
cases be treated as waste themselves (Palatsi et al., 2011). The cost for the
safe disposal of slaughterhouse waste in recent years has thus considerably
increased. This is primarily due to health risks from the presence of pathogens
in such wastes. Several different possibilities for their disposal exist, as
described in this review.
Composting is one alternative for the disposal of slaughterhouse wastes.
The process has various benefits, including reduced environmental pollution, the
generation of a valuable byproduct, and the destruction of a majority of
pathogens (NABC, 2004). The successful conversion of such wastes into
good-quality compost however requires close control. When performed under
stringent management, the final product should not pose a risk to animal and
human health (Gale, 2004). There are however some pathogens that are not able to
be destroyed by composting, such as prions and spore forming bacteria.
The process of AH of slaughterhouse wastes is relatively new. It uses a
strong base, heat and temperature to catalyze the hydrolysis of biological
materials into a sterile aqueous solution consisting of peptides, amino acids,
sugars and soaps (Kaye et al., 1998). This effluent is highly alkaline and very
rich in nutrients, and although it can be released into a sanitary sewer, it can
also potentially pose problems (NABC, 2004). It has been found to be extremely
effective in the elimination of many pathogens and prions from carcasses as well
as from animal wastes. The waste from the process is however very rich in
nutrients, and would thus offer high biogas generation potential.
AD is today one of the most promising methods for the disposal of
slaughterhouse waste (Gwyther et al., 2011). This process not only produces a
digestate which can be used as a valuable fertilizer, but it also produces heat
and biogas, that in turn can be converted to energy. Moreover, slaughterhouse
wastes are rich in proteins and nitrogen, and thus are ideal substrates for the
AD process. Numerous studies have reported various levels of effectiveness in
the removal of different pathogens using AD.
The results of our extensive literature review concerning the survival of
pathogens after composting, AH and AD are summarized in Table 1. Although there
would not appear to be a single approach that would inactivate all the pathogens
investigated in this study, an AD process with either a pre- or post-
pasteurization step would most likely inactivate the majority of microorganisms.
Prions would however survive a pasteurization and an AD process, as would
spore-forming bacteria. *** The survival of prions should however not be a cause
for concern, as any biogas plant operator should be able to prevent diseased
animals or suspected TSE diseased animals from entering the process.
...snip...end
>>>*** The survival of prions should however not be a cause for
concern, as any biogas plant operator should be able to prevent diseased animals
or suspected TSE diseased animals from entering the process.<<<
LMAO!!!...LOL!...on a wing and a prayer...tss
What is the risk of chronic wasting disease being introduced into Great
Britain? An updated Qualitative Risk Assessment March 2016
Summary
The previous assessment concentrated on the incursion of disease from North
America through the imports of animal feed or the movement of contaminated
clothing, footwear and equipment. The results suggested that import of pet feed
was a non-negligible risk, but given the unlikely contact of resident deer in GB
with such non-ruminant feed, this was considered overall a negligible to very
low risk. The movement of contaminated clothing, footwear or equipment
(particularly hunting equipment) could pose a very low risk, although the volume
of contaminated soil which would need to be ingested to give rise to an
infection is likely to be higher than would be present. There is a variable
level uncertainty in all these assessments.
The new assessment focuses on an additional potential route of entry: the
importation of natural deer urine lures. The main conclusions from this
assessment are:
In areas of North America where CWD has been reported, given that CWD is
excreted in faeces, saliva, urine and blood, and survives in the environment for
several years there is a medium probability that the deer urine in North America
contains CWD (high uncertainty; depends on the source of deer used for
production).
The risk of a deer in GB being infected per 30 ml bottle of urine imported
from the USA is very low, albeit with high uncertainty. Overall it is concluded
that the risk of at least one infection of deer in the UK with CWD per year from
deer urine lures imported from the USA is medium. This assumes a high number of
30 ml bottles imported per year from all areas of the USA.
None of the species affected by CWD in North America are present in GB. For
a British species to become infected with CWD following exposure, the dose and
inherent susceptibility of the species will be important. Based on current
scientific evidence Red deer (Cervus elaphus elaphus) are susceptible to CWD,
Fallow deer (Dama dama) are likely to be less susceptible and Roe deer
(Capreolus capreolus) have a gene conferring susceptibility. Therefore, it is
likely that given exposure to an infectious dose of CWD, deer in GB could become
infected with CWD.
Overall, the probability of importing CWD into GB from North America and
causing infection in British deer is uncertain but likely to be negligible to
very low via movement of deer hunters, other tourists and British servicemen and
very low via imported (non-
2
ruminant) animal feed and medium for the use of lures. However, if it was
imported and (a) deer did become infected with CWD, the consequences would be
severe as eradication of the disease is impossible, it is clinically
indistinguishable from BSE infection in deer (Dalgleish et al., 2008) and
populations of wild and farmed deer would be under threat.
The USA has implemented a Herd Certification Programme for farmed and
captive cervids. So far, 29 States are approved for HCP status (APHIS, 2015).
The list includes States such as Colorado, where CWD is present, therefore it is
recommended that any sourcing of such natural urine lures should be not only
from States with an HCP programme, but also from a herd which is registered as
being regularly tested free of CWD.
Animal urine is not considered a commodity which is subject to animal
by-products legislation for imports. Internet sales are common and although a
license would be required, there are no conditions for the safe sourcing of such
products. Deer urine lures are also available in Europe and may be produced from
carcases of hunted deer. The use of deer urine produced from a species not
present in Europe (such as white tailed deer) is questioned for its value with
native GB deer according to the British Deer Society survey.
Background
Thursday, April 07, 2016
What is the risk of chronic wasting disease being introduced into Great
Britain? An updated Qualitative Risk Assessment March 2016
Friday, December 14, 2012
DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced
into Great Britain? A Qualitative Risk Assessment October 2012
snip...
In the USA, under the Food and Drug Administration’s BSE Feed Regulation
(21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin)
from deer and elk is prohibited for use in feed for ruminant animals. With
regards to feed for non-ruminant animals, under FDA law, CWD positive deer may
not be used for any animal feed or feed ingredients. For elk and deer considered
at high risk for CWD, the FDA recommends that these animals do not enter the
animal feed system. However, this recommendation is guidance and not a
requirement by law.
Animals considered at high risk for CWD include:
1) animals from areas declared to be endemic for CWD and/or to be CWD
eradication zones and
2) deer and elk that at some time during the 60-month period prior to
slaughter were in a captive herd that contained a CWD-positive animal.
Therefore, in the USA, materials from cervids other than CWD positive
animals may be used in animal feed and feed ingredients for non-ruminants.
The amount of animal PAP that is of deer and/or elk origin imported from
the USA to GB can not be determined, however, as it is not specified in TRACES.
It may constitute a small percentage of the 8412 kilos of non-fish origin
processed animal proteins that were imported from US into GB in 2011.
Overall, therefore, it is considered there is a __greater than negligible
risk___ that (nonruminant) animal feed and pet food containing deer and/or elk
protein is imported into GB.
There is uncertainty associated with this estimate given the lack of data
on the amount of deer and/or elk protein possibly being imported in these
products.
snip...
36% in 2007 (Almberg et al., 2011). In such areas, population declines of
deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of
Colorado, the prevalence can be as high as 30% (EFSA, 2011).
The clinical signs of CWD in affected adults are weight loss and
behavioural changes that can span weeks or months (Williams, 2005). In addition,
signs might include excessive salivation, behavioural alterations including a
fixed stare and changes in interaction with other animals in the herd, and an
altered stance (Williams, 2005). These signs are indistinguishable from cervids
experimentally infected with bovine spongiform encephalopathy (BSE).
Given this, if CWD was to be introduced into countries with BSE such as GB,
for example, infected deer populations would need to be tested to differentiate
if they were infected with CWD or BSE to minimise the risk of BSE entering the
human food-chain via affected venison.
snip...
The rate of transmission of CWD has been reported to be as high as 30% and
can approach 100% among captive animals in endemic areas (Safar et al., 2008).
snip...
In summary, in endemic areas, there is a medium probability that the soil
and surrounding environment is contaminated with CWD prions and in a
bioavailable form. In rural areas where CWD has not been reported and deer are
present, there is a greater than negligible risk the soil is contaminated with
CWD prion.
snip...
In summary, given the volume of tourists, hunters and servicemen moving
between GB and North America, the probability of at least one person travelling
to/from a CWD affected area and, in doing so, contaminating their clothing,
footwear and/or equipment prior to arriving in GB is greater than negligible.
For deer hunters, specifically, the risk is likely to be greater given the
increased contact with deer and their environment. However, there is significant
uncertainty associated with these estimates.
snip...
Therefore, it is considered that farmed and park deer may have a higher
probability of exposure to CWD transferred to the environment than wild deer
given the restricted habitat range and higher frequency of contact with tourists
and returning GB residents.
snip...
What is the risk of chronic wasting disease being introduced into Great
Britain? A Qualitative Risk Assessment October 2012
I strenuously once again urge the FDA and its industry constituents, to
make it MANDATORY that all ruminant feed be banned to all ruminants, and this
should include all cervids, as well as non-ruminants such as cats and dogs as
well, as soon as possible for the following reasons...
31 Jan 2015 at 20:14 GMT
*** Ruminant feed ban for cervids in the United States? ***
31 Jan 2015 at 20:14 GMT
Terry Singeltary Sr. comment ;
Tuesday, April 19, 2016
Docket No. FDA-2013-N-0764 for Animal Feed Regulatory Program Standards
Singeltary Comment Submission
Thursday, August 25, 2016
FSIS Green Bay Dressed Beef Recalls Beef Products Due To Possible Specified
Risk Materials Contamination the most high risk materials for BSE TSE PRION AKA
MAD COW TYPE DISEASE
In the USA, USDA et al sometimes serves SRM’s up as appetizers or
horderves.
Monday, June 20, 2016
*** Specified Risk Materials SRMs BSE TSE Prion Program
Saturday, January 31, 2015
RAPID ADVICE 17-2014 : Evaluation of the risk for public health of casings
in countries with a “negligible risk status for BSE” and on the risk of
modification of the list of specified risk materials (SRM) with regard to BSE
*** Infectious agent of sheep scrapie may persist in the environment for at
least 16 years ***
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3
CWD TSE PRION HUMAN ZOONOSIS POTENTIAL, has it already happened, and being
masked as sporadic CJD? and what about iatrogenic, or the pass if forward,
friendly fire mode of transmission of cwd to humans, same thing, sporadic cjd ?
*** WDA 2016 NEW YORK ***
We found that CWD adapts to a new host more readily than BSE and that human
PrP was unexpectedly prone to misfolding by CWD prions. In addition, we
investigated the role of specific regions of the bovine, deer and human PrP
protein in resistance to conversion by prions from another species. We have
concluded that the human protein has a region that confers unusual
susceptibility to conversion by CWD prions.
Student Presentations Session 2
The species barriers and public health threat of CWD and BSE prions
Ms. Kristen Davenport1, Dr. Davin Henderson1, Dr. Candace Mathiason1, Dr.
Edward Hoover1 1Colorado State University
Chronic wasting disease (CWD) is spreading rapidly through cervid
populations in the USA. Bovine spongiform encephalopathy (BSE, mad cow disease)
arose in the 1980s because cattle were fed recycled animal protein. These and
other prion diseases are caused by abnormal folding of the normal prion protein
(PrP) into a disease causing form (PrPd), which is pathogenic to nervous system
cells and can cause subsequent PrP to misfold. CWD spreads among cervids very
efficiently, but it has not yet infected humans. On the other hand, BSE was
spread only when cattle consumed infected bovine or ovine tissue, but did infect
humans and other species. The objective of this research is to understand the
role of PrP structure in cross-species infection by CWD and BSE. To study the
propensity of each species’ PrP to be induced to misfold by the presence of PrPd
from verious species, we have used an in vitro system that permits detection of
PrPd in real-time. We measured the conversion efficiency of various combinations
of PrPd seeds and PrP substrate combinations. We observed the cross-species
behavior of CWD and BSE, in addition to feline-adapted CWD and BSE. We found
that CWD adapts to a new host more readily than BSE and that human PrP was
unexpectedly prone to misfolding by CWD prions. In addition, we investigated the
role of specific regions of the bovine, deer and human PrP protein in resistance
to conversion by prions from another species. We have concluded that the human
protein has a region that confers unusual susceptibility to conversion by CWD
prions. CWD is unique among prion diseases in its rapid spread in natural
populations. BSE prions are essentially unaltered upon passage to a new species,
while CWD adapts to the new species. This adaptation has consequences for
surveillance of humans exposed to CWD.
Wildlife Disease Risk Communication Research Contributes to Wildlife Trust
Administration Exploring perceptions about chronic wasting disease risks among
wildlife and agriculture professionals and stakeholders
Ms. Alyssa Wetterau1, Dr. Krysten Schuler1, Dr. Elizabeth Bunting1, Dr.
Hussni Mohammed1 1Cornell University
Chronic wasting disease (CWD) is a fatal disease of North American
Cervidae. New York State (NYS, USA) successfully managed an outbreak of CWD in
2005 in both captive and wild white-tailed deer (Odocoileus virginianus) with no
reoccurrence of the disease as of 2015. To attain maximum compliance and
efficacy of management actions for prevention of CWD entry, understanding the
varied risk perceptions will allow for targeted, proactive communication efforts
to address divergences between expert-derived risk assessments and stakeholder
risk perceptions. We examined perceived risks associated with CWD introduction
and exposure among agricultural and wildlife agency professionals within and
outside of NYS, as well as stakeholder groups (e.g., hunters and captive cervid
owners). We measured perceived risk using a risk assessment questionnaire online
via Qualtrics survey software and evaluated similarities within, as well as
differences in, perception among participant groups. New York State biologists
employed by the Department of Environmental Conservation and independent non-NYS
wildlife and agricultural professionals thought CWD risks associated with
captive cervids were high; captive cervid owners thought risks for wild and
captive cervids were low. Agriculture and wildlife professional groups agreed on
general risk perceptions. We ranked 15 individual risk hazards into high and low
medium categories based on all responses. Differences between groups were most
evident in hypothetical disease pathways. Any pathway involving inter-state
import of live cervids received high ranking for all groups except captive
cervid owners. Comparatively low risk perceptions by captive cervid operators
may stem from misinformation, lack of understanding of testing programs, and
indemnity payments for animal depopulation. Communication and education directed
at areas of disagreement may facilitate effective disease prevention and
management.
* No evaluation of determination of CWD risk is required for alternative
livestock or captive wildlife shipped directly to slaughter or to a biosecure
facility approved by the Division and the Dept. of Agriculture.
*** We found that CWD adapts to a new host more readily than BSE and that
human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we
investigated the role of specific regions of the bovine, deer and human PrP
protein in resistance to conversion by prions from another species. We have
concluded that the human protein has a region that confers unusual
susceptibility to conversion by CWD prions. CWD is unique among prion diseases
in its rapid spread in natural populations. BSE prions are essentially unaltered
upon passage to a new species, while CWD adapts to the new species. This
adaptation has consequences for surveillance of humans exposed to CWD. ***
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
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
*** PPo3-7: Prion Transmission from Cervids to Humans is Strain-dependent
*** Here we report that a human prion strain that had adopted the cervid
prion protein (PrP) sequence through passage in cervidized transgenic mice
efficiently infected transgenic mice expressing human PrP,
*** indicating that the species barrier from cervid to humans is prion
strain-dependent and humans can be vulnerable to novel cervid prion strains.
PPo2-27:
Generation of a Novel form of Human PrPSc by Inter-species Transmission of
Cervid Prions
*** Our findings suggest that CWD prions have the capability to infect
humans, and that this ability depends on CWD strain adaptation, implying that
the risk for human health progressively increases with the spread of CWD among
cervids.
PPo2-7:
Biochemical and Biophysical Characterization of Different CWD Isolates
*** The data presented here substantiate and expand previous reports on the
existence of different CWD strains.
Envt.07:
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free
Ranging White-Tailed Deer Infected with Chronic Wasting Disease
***The presence and seeding activity of PrPTSE in skeletal muscle from
CWD-infected cervids suggests prevention of such tissue in the human diet as a
precautionary measure for food safety, pending on further clarification of
whether CWD may be transmissible to humans.
>>>CHRONIC WASTING DISEASE , THERE WAS NO ABSOLUTE BARRIER TO
CONVERSION OF THE HUMAN PRION PROTEIN<<<
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
Wednesday, January 01, 2014
Molecular Barriers to Zoonotic Transmission of Prions
*** 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.
Envt.07:
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free
Ranging White-Tailed Deer Infected with Chronic Wasting Disease
***The presence and seeding activity of PrPTSE in skeletal muscle from
CWD-infected cervids suggests prevention of such tissue in the human diet as a
precautionary measure for food safety, pending on further clarification of
whether CWD may be transmissible to humans.
Yet, it has to be noted that our assessments of PrPTSE levels in skeletal
muscles were based on findings in presumably pre- or subclinically infected
animals. Therefore, the concentration of PrPTSE in skeletal muscles of WTD with
clinically manifest CWD may possibly exceed our estimate which refers to
clinically inconspicuous animals that are more likely to enter the human food
chain. Our tissue blot findings in skeletal muscles from CWD-infected WTD would
be consistent with an anterograde spread of CWD prions via motor nerve fibres to
muscle tissue (figure 4A). Similar neural spreading pathways of muscle infection
were previously found in hamsters orally challenged with scrapie [28] and
suggested by the detection of PrPTSE in muscle fibres and muscle-associated
nerve fascicles of clinically-ill non-human primates challenged with BSE prions
[29]. Whether the absence of detectable PrPTSE in myofibers observed in our
study is a specific feature of CWD in WTD, or was due to a pre- or subclinical
stage of infection in the examined animals, remains to be established. In any
case, our observations support previous findings suggesting the precautionary
prevention of muscle tissue from CWD-infected WTD in the human diet, and
highlight the need to comprehensively elucidate of whether CWD may be
transmissible to humans. While the understanding of TSEs in cervids has made
substantial progress during the past few years, the assessment and management of
risks possibly emanating from prions in skeletal muscles of CWD-infected cervids
requires further research.
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease Rachel C.
Angers1,*, Shawn R. Browning1,*,†, Tanya S. Seward2, Christina J. Sigurdson4,‡,
Michael W. Miller5, Edward A. Hoover4, Glenn C. Telling1,2,3,§ + Author
Affiliations
1 Department of Microbiology, Immunology and Molecular Genetics, University
of Kentucky, Lexington, KY 40536, USA. 2 Sanders Brown Center on Aging,
University of Kentucky, Lexington, KY 40536, USA. 3 Department of Neurology,
University of Kentucky, Lexington, KY 40536, USA. 4 Department of Microbiology,
Immunology and Pathology, Colorado State University, Fort Collins, CO 80523,
USA. 5 Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO
80526, USA. ↵§ To whom correspondence should be addressed. E-mail:
gtell2@uky.edu ↵* These authors contributed equally to this work.
↵† Present address: Department of Infectology, Scripps Research Institute,
5353 Parkside Drive, RF-2, Jupiter, FL 33458, USA.
↵‡ Present address: Institute of Neuropathology, University of Zurich,
Schmelzbergstrasse 12, 8091 Zurich, Switzerland.
Abstract The emergence of chronic wasting disease (CWD) in deer and elk in
an increasingly wide geographic area, as well as the interspecies transmission
of bovine spongiform encephalopathy to humans in the form of variant Creutzfeldt
Jakob disease, have raised concerns about the zoonotic potential of CWD. Because
meat consumption is the most likely means of exposure, it is important to
determine whether skeletal muscle of diseased cervids contains prion
infectivity. Here bioassays in transgenic mice expressing cervid prion protein
revealed the presence of infectious prions in skeletal muscles of CWD-infected
deer, demonstrating that humans consuming or handling meat from CWD-infected
deer are at risk to prion exposure.
Exotic Meats USA Announces Urgent Statewide Recall of Elk Tenderloin
Because It May Contain Meat Derived From An Elk Confirmed To Have Chronic
Wasting Disease
Contact: Exotic Meats USA 1-800-680-4375
FOR IMMEDIATE RELEASE -- February 9, 2009 -- Exotic Meats USA of San
Antonio, TX is initiating a voluntary recall of Elk Tenderloin because it may
contain meat derived from an elk confirmed to have Chronic Wasting Disease
(CWD). The meat with production dates of December 29, 30 and 31, 2008 was
purchased from Sierra Meat Company in Reno, NV. The infected elk came from Elk
Farm LLC in Pine Island, MN and was among animals slaughtered and processed at
USDA facility Noah’s Ark Processors LLC.
Chronic Wasting Disease (CWD) is a fatal brain and nervous system disease
found in elk and deer. The disease is caused by an abnormally shaped protein
called a prion, which can damage the brain and nerves of animals in the deer
family. Currently, it is believed that the prion responsible for causing CWD in
deer and elk is not capable of infecting humans who eat deer or elk contaminated
with the prion, but the observation of animal-to-human transmission of other
prion-mediated diseases, such as bovine spongiform encephalopathy (BSE), has
raised a theoretical concern regarding the transmission of CWD from deer or elk
to humans. At the present time, FDA believes the risk of becoming ill from
eating CWD-positive elk or deer meat is remote. However, FDA strongly advises
consumers to return the product to the place of purchase, rather than disposing
of it themselves, due to environmental concerns.
Exotic Meats USA purchased 1 case of Elk Tenderloins weighing 16.9 lbs. The
Elk Tenderloin was sold from January 16 – 27, 2009. The Elk Tenderloins was
packaged in individual vacuum packs weighing approximately 3 pounds each. A
total of six packs of the Elk Tenderloins were sold to the public at the Exotic
Meats USA retail store. Consumers who still have the Elk Tenderloins should
return the product to Exotic Meats USA at 1003 NE Loop 410, San Antonio, TX
78209. Customers with concerns or questions about the Voluntary Elk Recall can
call 1-800-680-4375. The safety of our customer has always been and always will
be our number one priority.
Exotic Meats USA requests that for those customers who have products with
the production dates in question, do not consume or sell them and return them to
the point of purchase. Customers should return the product to the vendor. The
vendor should return it to the distributor and the distributor should work with
the state to decide upon how best to dispose. If the consumer is disposing of
the product he/she should consult with the local state EPA office.
#
COLORADO: Farmer's market meat recalled after testing positive for CWD
24.dec.08 9News.com Jeffrey Wolf
Elk meat that was sold at a farmer's market is being recalled because tests
show it was infected with chronic wasting disease. The Boulder County Health
Department and Colorado Department of Public Health and Environment issued the
recall Wednesday after the meat was sold at the Boulder County Fairgrounds on
Dec. 13. Although there isn't any human health risk connected with CWD, the
recalled was issued as a precaution. About 15 elk were bought from a commercial
ranch in Colorado in early December and processed at a licensed plant. All 15
were tested for CWD and one came up positive. The labeling on the product would
have the following information: *Seller: High Wire Ranch *The type of cut:
"chuck roast," "arm roast," "flat iron," "ribeye steak," "New York steak,"
"tenderloin," "sirloin tip roast," "medallions" or "ground meat." *Processor:
Cedaredge Processing *The USDA triangle containing the number "34645" People
with questions about this meat can contact John Pape, epidemiologist at the
Colorado Department of Public Health and Environment at 303-692-2628.
COULD NOT FIND any warning or recalls on these two sites confirming their
recall of CWD infected meat. ...TSS
Wednesday, April 06, 2011
Presence and Seeding Activity of Pathological Prion Protein (PrPTSE) in
Skeletal Muscles of White-Tailed Deer Infected with Chronic Wasting Disease
Prion Infectivity in Fat of Deer with Chronic Wasting Disease
Brent Race,# Kimberly Meade-White,# Richard Race, and Bruce Chesebro* Rocky
Mountain Laboratories, 903 South 4th Street, Hamilton, Montana 59840
Received 2 June 2009/ Accepted 24 June 2009
ABSTRACT Top ABSTRACT TEXT REFERENCES
Chronic wasting disease (CWD) is a neurodegenerative prion disease of
cervids. Some animal prion diseases, such as bovine spongiform encephalopathy,
can infect humans; however, human susceptibility to CWD is unknown. In
ruminants, prion infectivity is found in central nervous system and lymphoid
tissues, with smaller amounts in intestine and muscle. In mice, prion
infectivity was recently detected in fat. Since ruminant fat is consumed by
humans and fed to animals, we determined infectivity titers in fat from two
CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD
infectivity and might be a risk factor for prion infection of other species.
snip...
The highest risk of human contact with CWD might be through exposure to
high-titer CNS tissue through accidental skin cuts or corneal contact at the
time of harvest and butchering. However, the likelihood of a human consuming fat
infected with a low titer of the CWD agent is much higher. It is impossible to
remove all the fat present within muscle tissue, and fat consumption is
inevitable when eating meat. Of additional concern is the fact that meat from an
individual deer harvested by a hunter is typically consumed over multiple meals
by the same group of people. These individuals would thus have multiple
exposures to the CWD agent over time, which might increase the chance for
transfer of infection.
In the Rocky Mountain region of North America, wild deer are subject to
predation by wolves, coyotes, bears, and mountain lions. Although canines such
as wolves and coyotes are not known to be susceptible to prion diseases, felines
definitely are susceptible to BSE (9) and might also be infected by the CWD
agent. Deer infected with the CWD agent are more likely to be killed by
predators such as mountain lions (11). Peripheral tissues, including lymph
nodes, muscle, and fat, which harbor prion infectivity are more accessible for
consumption than CNS tissue, which has the highest level of infectivity late in
disease. Therefore, infectivity in these peripheral tissues may be important in
potential cross-species CWD transmissions in the wild.
The present finding of CWD infectivity in deer fat tissue raises the
possibility that prion infectivity might also be found in fat tissue of other
infected ruminants, such as sheep and cattle, whose fat and muscle tissues are
more widely distributed in both the human and domestic-animal food chains.
Although the infectivity in fat tissues is low compared to that in the CNS,
there may be significant differences among species and between prion strains.
Two fat samples from BSE agent-infected cattle were reported to be negative by
bioassay in nontransgenic RIII mice (3, 6). However, RIII mice are
10,000-fold-less sensitive to BSE agent infection than transgenic mice
expressing bovine PrP (4). It would be prudent to carry out additional
infectivity assays on fat from BSE agent-infected cattle and scrapie
agent-infected sheep using appropriate transgenic mice or homologous species to
determine the risk from these sources.
0C7.04
North American Cervids Harbor Two Distinct CWD Strains
Authors
Angers, R. Seward, T, Napier, D., Browning, S., Miller, M., Balachandran
A., McKenzie, D., Hoover, E., Telling, G. 'University of Kentucky; Colorado
Division of Wildlife, Canadian Food Inspection Agency; University Of Wisconsin;
Colorado State University.
Content
Despite the increasing geographic distribution and host range of CWD,
little is known about the prion strain(s) responsible for distinct outbreaks of
the disease. To address this we inoculated CWD-susceptible Tg(CerPrP)1536+/·
mice with 29 individual prion samples from various geographic locations in North
America. Upon serial passage, intrastudy incubation periods consistently
diverged and clustered into two main groups with means around 210 and 290 days,
with corresponding differences in neuropathology. Prion strain designations were
utilized to distinguish between the two groups: Type I CWD mice succumbed to
disease in the 200 day range and displayed a symmetrical pattern of vacuolation
and PrPSc deposition, whereas Type II CWD mice succumbed to disease near 300
days and displayed a strikingly different pattern characterized by large local
accumulations of florid plaques distributed asymmetrically. Type II CWD bears a
striking resemblance to unstable parental scrapie strains such as 87A which give
rise to stable, short incubation period strains such as ME7 under certain
passage conditions. In agreement, the only groups of CWD-inoculated mice with
unwavering incubation periods were those with Type I CWD. Additionally,
following endpoint titration of a CWD sample, Type I CWD could be recovered only
at the lowest dilution tested (10-1), whereas Type II CWD was detected in mice
inoculated with all dilutions resulting in disease. Although strain properties
are believed to be encoded in the tertiary structure of the infectious prion
protein, we found no biochemical differences between Type I and Type II CWD. Our
data confirm the co·existence of two distinct prion strains in CWD-infected
cervids and suggest that Type II CWD is the parent strain of Type I CWD.
see page 29, and see other CWD studies ;
Sunday, November 23, 2008
PRION October 8th - 10th 2008 Book of Abstracts
ADAPTATION OF CHRONIC WASTING DISEASE (CWD) INTO HAMSTERS, EVIDENCE OF A
WISCONSIN STRAIN OF CWD
Chad Johnson1, Judd Aiken2,3,4 and Debbie McKenzie4,5 1 Department of
Comparative Biosciences, University of Wisconsin, Madison WI, USA 53706 2
Department of Agriculture, Food and Nutritional Sciences, 3 Alberta Veterinary
Research Institute, 4.Center for Prions and Protein Folding Diseases, 5
Department of Biological Sciences, University of Alberta, Edmonton AB, Canada
T6G 2P5
The identification and characterization of prion strains is increasingly
important for the diagnosis and biological definition of these infectious
pathogens. Although well-established in scrapie and, more recently, in BSE,
comparatively little is known about the possibility of prion strains in chronic
wasting disease (CWD), a disease affecting free ranging and captive cervids,
primarily in North America. We have identified prion protein variants in the
white-tailed deer population and demonstrated that Prnp genotype affects the
susceptibility/disease progression of white-tailed deer to CWD agent. The
existence of cervid prion protein variants raises the likelihood of distinct CWD
strains. Small rodent models are a useful means of identifying prion strains. We
intracerebrally inoculated hamsters with brain homogenates and phosphotungstate
concentrated preparations from CWD positive hunter-harvested (Wisconsin CWD
endemic area) and experimentally infected deer of known Prnp genotypes. These
transmission studies resulted in clinical presentation in primary passage of
concentrated CWD prions. Subclinical infection was established with the other
primary passages based on the detection of PrPCWD in the brains of hamsters and
the successful disease transmission upon second passage. Second and third
passage data, when compared to transmission studies using different CWD inocula
(Raymond et al., 2007) indicate that the CWD agent present in the Wisconsin
white-tailed deer population is different than the strain(s) present in elk,
mule-deer and white-tailed deer from the western United States endemic region.
Tuesday, December 16, 2014
Evidence for zoonotic potential of ovine scrapie prions
Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves
Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle
Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia
Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1,
Affiliations Contributions Corresponding author Journal name: Nature
Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821
Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014
Article tools Citation Reprints Rights & permissions Article metrics
Abstract
Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant
Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie
prions remains unknown. Mice genetically engineered to overexpress the human
prion protein (tgHu) have emerged as highly relevant models for gauging the
capacity of prions to transmit to humans. These models can propagate human
prions without any apparent transmission barrier and have been used used to
confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie
prions transmit to several tgHu mice models with an efficiency comparable to
that of cattle BSE. The serial transmission of different scrapie isolates in
these mice led to the propagation of prions that are phenotypically identical to
those causing sporadic CJD (sCJD) in humans. These results demonstrate that
scrapie prions have a zoonotic potential and raise new questions about the
possible link between animal and human prions.
Subject terms: Biological sciences• Medical research At a glance
*** 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.
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 efficiency comparable to that of cattle BSE. While the
efficiency 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
1978 SCRAPIE IN CONFIDENCE SCJD
1979
SILENCE ON CJD AND SCRAPIE
1980
SILENCE ON CJD AND SCRAPIE
*** 1981 NOVEMBER
Thursday, August 04, 2016
*** MEETING ON THE FEASIBILITY OF CARRYING OUT EPIDEMIOLOGICAL STUDIES ON
CREUTZFELDT JAKOB DISEASE 1978 THE SCRAPIE FILES IN CONFIDENCE CONFIDENTIAL SCJD
2016
SCRAPIE AND CWD ZOONOSIS
PRION 2016 CONFERENCE 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
Transmission of scrapie prions to primate after an extended silent
incubation period
***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.***
Transmission of scrapie prions to primate after an extended silent
incubation period
Emmanuel E. Comoy , Jacqueline Mikol , Sophie Luccantoni-Freire , Evelyne
Correia , Nathalie Lescoutra-Etchegaray , Valérie Durand , Capucine Dehen ,
Olivier Andreoletti , Cristina Casalone , Juergen A. Richt , Justin J. Greenlee
, Thierry Baron , Sylvie L. Benestad , Paul Brown & Jean-Philippe Deslys
Abstract
Classical bovine spongiform encephalopathy (c-BSE) is the only animal prion
disease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD)
in humans and having guided protective measures for animal and human health
against animal prion diseases. Recently, partial transmissions to humanized mice
showed that the zoonotic potential of scrapie might be similar to c-BSE. We here
report the direct transmission of a natural classical scrapie isolate to
cynomolgus macaque, a highly relevant model for human prion diseases, after a
10-year silent incubation period, with features similar to those reported for
human cases of sporadic CJD. Scrapie is thus actually transmissible to primates
with incubation periods compatible with their life expectancy, although fourfold
longer than BSE. Long-term experimental transmission studies are necessary to
better assess the zoonotic potential of other prion diseases with high
prevalence, notably Chronic Wasting Disease of deer and elk and atypical/Nor98
scrapie.
snip...
In addition to previous studies on scrapie transmission to primate1,8,9 and
the recently published study on transgenic humanized mice13, our results
constitute new evidence for recommending that the potential risk of scrapie for
human health should not be dismissed. Indeed, human PrP transgenic mice and
primates are the most relevant models for investigating the human transmission
barrier. To what extent such models are informative for measuring the zoonotic
potential of an animal TSE under field exposure conditions is unknown. During
the past decades, many protective measures have been successfully implemented to
protect cattle from the spread of c-BSE, and some of these measures have been
extended to sheep and goats to protect from scrapie according to the principle
of precaution. Since cases of c-BSE have greatly reduced in number, those
protective measures are currently being challenged and relaxed in the absence of
other known zoonotic animal prion disease. We recommend that risk managers
should be aware of the long term potential risk to human health of at least
certain scrapie isolates, notably for lymphotropic strains like the classical
scrapie strain used in the current study. Relatively high amounts of infectivity
in peripheral lymphoid organs in animals infected with these strains could lead
to contamination of food products produced for human consumption. Efforts should
also be maintained to further assess the zoonotic potential of other animal
prion strains in long-term studies, notably lymphotropic strains with high
prevalence like CWD, which is spreading across North America, and atypical/Nor98
scrapie (Nor98)50 that was first detected in the past two decades and now
represents approximately half of all reported cases of prion diseases in small
ruminants worldwide, including territories previously considered as scrapie
free. Even if the prevailing view is that sporadic CJD is due to the spontaneous
formation of CJD prions, it remains possible that its apparent sporadic nature
may, at least in part, result from our limited capacity to identify an
environmental origin.
***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.***
2015
O.05: Transmission of prions to primates after extended silent incubation
periods: Implications for BSE and scrapie risk assessment in human populations
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni,
Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys
Atomic Energy Commission; Fontenay-aux-Roses, France
Prion diseases (PD) are the unique neurodegenerative proteinopathies
reputed to be transmissible under field conditions since decades. The
transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that
an animal PD might be zoonotic under appropriate conditions. Contrarily, in the
absence of obvious (epidemiological or experimental) elements supporting a
transmission or genetic predispositions, PD, like the other proteinopathies, are
reputed to occur spontaneously (atpical animal prion strains, sporadic CJD
summing 80% of human prion cases). Non-human primate models provided the first
evidences supporting the transmissibiity of human prion strains and the zoonotic
potential of BSE. Among them, cynomolgus macaques brought major information for
BSE risk assessment for human health (Chen, 2014), according to their
phylogenetic proximity to humans and extended lifetime. We used this model to
assess the zoonotic potential of other animal PD from bovine, ovine and cervid
origins even after very long silent incubation periods.
*** We recently observed the direct transmission of a natural classical
scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD,
albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked
in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases. We will present an
updated panorama of our different transmission studies and discuss the
implications of such extended incubation periods on risk assessment of animal PD
for human health.
===============
***thus questioning the origin of human sporadic cases***
===============
***our findings suggest that possible transmission risk of H-type BSE to
sheep and human. Bioassay will be required to determine whether the PMCA
products are infectious to these animals.
==============
Saturday, May 28, 2016
*** Infection and detection of PrPCWD in soil from CWD infected farm in
Korea Prion 2016 Tokyo ***
What is the risk of chronic wasting disease being introduced into Great
Britain? An updated Qualitative Risk Assessment March 2016
Summary
The previous assessment concentrated on the incursion of disease from North
America through the imports of animal feed or the movement of contaminated
clothing, footwear and equipment. The results suggested that import of pet feed
was a non-negligible risk, but given the unlikely contact of resident deer in GB
with such non-ruminant feed, this was considered overall a negligible to very
low risk. The movement of contaminated clothing, footwear or equipment
(particularly hunting equipment) could pose a very low risk, although the volume
of contaminated soil which would need to be ingested to give rise to an
infection is likely to be higher than would be present. There is a variable
level uncertainty in all these assessments.
The new assessment focuses on an additional potential route of entry: the
importation of natural deer urine lures. The main conclusions from this
assessment are:
In areas of North America where CWD has been reported, given that CWD is
excreted in faeces, saliva, urine and blood, and survives in the environment for
several years there is a medium probability that the deer urine in North America
contains CWD (high uncertainty; depends on the source of deer used for
production).
The risk of a deer in GB being infected per 30 ml bottle of urine
imported from the USA is very low, albeit with high uncertainty. Overall it is
concluded that the risk of at least one infection of deer in the UK with CWD per
year from deer urine lures imported from the USA is medium. This assumes a high
number of 30 ml bottles imported per year from all areas of the USA.
None of the species affected by CWD in North America are present in GB.
For a British species to become infected with CWD following exposure, the dose
and inherent susceptibility of the species will be important. Based on current
scientific evidence Red deer (Cervus elaphus elaphus) are susceptible to CWD,
Fallow deer (Dama dama) are likely to be less susceptible and Roe deer
(Capreolus capreolus) have a gene conferring susceptibility. Therefore, it is
likely that given exposure to an infectious dose of CWD, deer in GB could become
infected with CWD.
Overall, the probability of importing CWD into GB from North America and
causing infection in British deer is uncertain but likely to be negligible to
very low via movement of deer hunters, other tourists and British servicemen and
very low via imported (non-
2
ruminant) animal feed and medium for the use of lures. However, if it was
imported and (a) deer did become infected with CWD, the consequences would be
severe as eradication of the disease is impossible, it is clinically
indistinguishable from BSE infection in deer (Dalgleish et al., 2008) and
populations of wild and farmed deer would be under threat.
The USA has implemented a Herd Certification Programme for farmed and
captive cervids. So far, 29 States are approved for HCP status (APHIS, 2015).
The list includes States such as Colorado, where CWD is present, therefore it is
recommended that any sourcing of such natural urine lures should be not only
from States with an HCP programme, but also from a herd which is registered as
being regularly tested free of CWD.
Animal urine is not considered a commodity which is subject to animal
by-products legislation for imports. Internet sales are common and although a
license would be required, there are no conditions for the safe sourcing of such
products. Deer urine lures are also available in Europe and may be produced from
carcases of hunted deer. The use of deer urine produced from a species not
present in Europe (such as white tailed deer) is questioned for its value with
native GB deer according to the British Deer Society survey.
Background
Thursday, April 07, 2016
What is the risk of chronic wasting disease being introduced into Great
Britain? An updated Qualitative Risk Assessment March 2016
Friday, December 14, 2012
DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced
into Great Britain? A Qualitative Risk Assessment October 2012
snip...
In the USA, under the Food and Drug Administration’s BSE Feed Regulation
(21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin)
from deer and elk is prohibited for use in feed for ruminant animals. With
regards to feed for non-ruminant animals, under FDA law, CWD positive deer may
not be used for any animal feed or feed ingredients. For elk and deer considered
at high risk for CWD, the FDA recommends that these animals do not enter the
animal feed system. However, this recommendation is guidance and not a
requirement by law.
Animals considered at high risk for CWD include:
1) animals from areas declared to be endemic for CWD and/or to be CWD
eradication zones and
2) deer and elk that at some time during the 60-month period prior to
slaughter were in a captive herd that contained a CWD-positive animal.
Therefore, in the USA, materials from cervids other than CWD positive
animals may be used in animal feed and feed ingredients for non-ruminants.
The amount of animal PAP that is of deer and/or elk origin imported from
the USA to GB can not be determined, however, as it is not specified in TRACES.
It may constitute a small percentage of the 8412 kilos of non-fish origin
processed animal proteins that were imported from US into GB in 2011.
Overall, therefore, it is considered there is a __greater than negligible
risk___ that (nonruminant) animal feed and pet food containing deer and/or elk
protein is imported into GB.
There is uncertainty associated with this estimate given the lack of data
on the amount of deer and/or elk protein possibly being imported in these
products.
snip...
36% in 2007 (Almberg et al., 2011). In such areas, population declines of
deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of
Colorado, the prevalence can be as high as 30% (EFSA, 2011).
The clinical signs of CWD in affected adults are weight loss and
behavioural changes that can span weeks or months (Williams, 2005). In addition,
signs might include excessive salivation, behavioural alterations including a
fixed stare and changes in interaction with other animals in the herd, and an
altered stance (Williams, 2005). These signs are indistinguishable from cervids
experimentally infected with bovine spongiform encephalopathy (BSE).
Given this, if CWD was to be introduced into countries with BSE such as GB,
for example, infected deer populations would need to be tested to differentiate
if they were infected with CWD or BSE to minimise the risk of BSE entering the
human food-chain via affected venison.
snip...
The rate of transmission of CWD has been reported to be as high as 30% and
can approach 100% among captive animals in endemic areas (Safar et al., 2008).
snip...
In summary, in endemic areas, there is a medium probability that the soil
and surrounding environment is contaminated with CWD prions and in a
bioavailable form. In rural areas where CWD has not been reported and deer are
present, there is a greater than negligible risk the soil is contaminated with
CWD prion.
snip...
In summary, given the volume of tourists, hunters and servicemen moving
between GB and North America, the probability of at least one person travelling
to/from a CWD affected area and, in doing so, contaminating their clothing,
footwear and/or equipment prior to arriving in GB is greater than negligible.
For deer hunters, specifically, the risk is likely to be greater given the
increased contact with deer and their environment. However, there is significant
uncertainty associated with these estimates.
snip...
Therefore, it is considered that farmed and park deer may have a higher
probability of exposure to CWD transferred to the environment than wild deer
given the restricted habitat range and higher frequency of contact with tourists
and returning GB residents.
snip...
What is the risk of chronic wasting disease being introduced into Great
Britain? A Qualitative Risk Assessment October 2012
I strenuously once again urge the FDA and its industry constituents, to
make it MANDATORY that all ruminant feed be banned to all ruminants, and this
should include all cervids, as well as non-ruminants such as cats and dogs as
well, as soon as possible for the following reasons...
31 Jan 2015 at 20:14 GMT
*** Ruminant feed ban for cervids in the United States? ***
31 Jan 2015 at 20:14 GMT
Terry Singeltary Sr. comment ;
Tuesday, April 19, 2016
Docket No. FDA-2013-N-0764 for Animal Feed Regulatory Program Standards
Singeltary Comment Submission
*** Infectious agent of sheep scrapie may persist in the environment for at
least 16 years ***
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3
Friday, August 14, 2015
*** Susceptibility of cattle to the agent of chronic wasting disease from
elk after intracranial inoculation ***
Saturday, May 28, 2016
*** Infection and detection of PrPCWD in soil from CWD infected farm in
Korea Prion 2016 Tokyo ***
Experimental Oral Transmission of Chronic Wasting Disease to Reindeer
(Rangifer tarandus tarandus)
Gordon B. Mitchell1, Christina J. Sigurdson2,3, Katherine I. O’Rourke4,
James Algire1, Noel P. Harrington1, Ines Walther1, Terry R. Spraker5, Aru
Balachandran1*
1 National and OIE Reference Laboratory for Scrapie and CWD, Canadian Food
Inspection Agency, Ottawa Laboratory – Fallowfield, Ottawa, Ontario, Canada,
2 Departments of Pathology and Medicine, University of California, San
Diego, La Jolla, California, United States of America, 3 Department of
Pathology, Microbiology and Immunology, University of California, Davis,
California, United States of America, 4 Animal Disease Research Unit,
Agricultural Research Service, United States Department of Agriculture, Pullman,
Washington, United States of America, 5 Veterinary Diagnostic Laboratory,
Colorado State University, Fort Collins, Colorado, United States of America
Abstract
Chronic wasting disease (CWD), a transmissible spongiform encephalopathy of
cervids, remains prevalent in North American elk, white-tailed deer and mule
deer. A natural case of CWD in reindeer (Rangifer tarandus tarandus) has not
been reported despite potential habitat overlap with CWD-infected deer or elk
herds. This study investigates the experimental transmission of CWD from elk or
white-tailed deer to reindeer by the oral route of inoculation. Ante-mortem
testing of the three reindeer exposed to CWD from white-tailed deer identified
the accumulation of pathological PrP (PrPCWD) in the recto-anal mucosa
associated lymphoid tissue (RAMALT) of two reindeer at 13.4 months
post-inoculation. Terminal CWD occurred in the two RAMALT-positive reindeer at
18.5 and 20 months post-inoculation while one other reindeer in the white-tailed
deer CWD inoculum group and none of the 3 reindeer exposed to elk CWD developed
disease. Tissue distribution analysis of PrPCWD in CWD-affected reindeer
revealed widespread deposition in central and peripheral nervous systems,
lymphoreticular tissues, the gastrointestinal tract, neuroendocrine tissues and
cardiac muscle. Analysis of prion protein gene (PRNP) sequences in the 6
reindeer identified polymorphisms at residues 2 (V/M), 129 (G/S), 138 (S/N) and
169 (V/M). These findings demonstrate that (i) a sub-population of reindeer are
susceptible to CWD by oral inoculation implicating the potential for
transmission to other Rangifer species, and (ii) certain reindeer PRNP
polymorphisms may be protective against CWD infection.
snip...
The importance of Rangifer species to the culture of aboriginal peoples
cannot be underestimated with many components of hunted animals being consumed
as food. Although relatively limited in comparison to elk and deer industries in
North America, reindeer and caribou farming does occur, producing consumables
such as meat, hides and antler velvet. The human health risks of consuming meat
or other products derived from CWD-infected animals remain uncertain, although
epidemiological evidence indicates transmission has not yet occurred [30,31,32]
and transgenic mouse studies suggest the risk is remote in humans expressing
common PRNP sequences [33,34,35]. The finding that CWD can be transmitted to
squirrel monkeys by intracranial inoculation [36] raises concern for human
transmissibility, however a study in macaques failed to demonstrate transmission
after 70 months [37]. Since prion strains may undergo changes in transmission
characteristics following passage through different species and strain selection
pressures can be exerted by host genetic factors during passage within a species
[24,38,39,40], caution is warranted when predicting the risks of CWD
transmission from reindeer to other species.
This is the first evidence of CWD transmission to the sub-species Rangifer
tarandus tarandus, implicating the potential for transmission to others in this
genus. Current diagnostic tests, including antemortem RAMALT testing, appear
capable of detecting CWD in Rangifer species and increased surveillance would be
required to determine if natural transmission has indeed occurred. Additional
studies are ongoing to chart the distribution of infectivity during the course
of disease and determine the influence of PRNP polymorphisms in disease
susceptibility.
Citation: Mitchell GB, Sigurdson CJ, O’Rourke KI, Algire J, Harrington NP,
et al. (2012) Experimental Oral Transmission of Chronic Wasting Disease to
Reindeer (Rangifer tarandus tarandus). PLoS ONE 7(6): e39055.
doi:10.1371/journal.pone.0039055
Editor: Anthony E. Kincaid, Creighton University, United States of America
Received May 4, 2012; Accepted May 15, 2012; Published June 18, 2012
This is an open-access article, free of all copyright, and may be freely
reproduced, distributed, transmitted, modified, built upon, or otherwise used by
anyone for any lawful purpose. The work is made available under the Creative
Commons CC0 public domain dedication.
Funding: This work was supported by the Canadian Food Inspection Agency and
Agriculture and Agri-Food Canada under C00233, the United States
Department of Agriculture Agricultural Research Service under CRIS
5348-32000-026-00-D and the United States National Institutes of Health under
NS069566 and U54AI0359. The funders had no role in study design, data collection
and analysis, decision to publish, or preparation of the manuscript.
Competing Interests: The authors have declared that no competing interests
exist.
* E-mail: Aru.Balachandran@inspection.gc.ca
THANK YOU PLOS FOR OPEN ACCESS FOR THE LAYPEOPLE, such as myself. ...TSS
natural cases of CWD in eight Sika deer (Cervus nippon) and five Sika/red
deer crossbreeds Korea and Experimental oral transmission to red deer (Cervus
elaphus elaphus)
SCWDS BRIEFS
A Quarterly Newsletter from the Southeastern Cooperative Wildlife Disease
Study College of Veterinary Medicine The University of Georgia Athens, Georgia
30602
Volume 27 January 2012 Number 4
Red deer susceptibility to CWD via oral inoculation was demonstrated in a
study conducted by collaborators from the U.S. and Canada. Red deer developed
clinical signs and had spongiform changes in the brain when euthanatized at 20
MPI. The CWD prion was detectable in neural and lymphoid tissues, endocrine
organs, cardiac muscle, nasal mucosa, and other tissues. Although field cases of
CWD in red deer have not been reported, results of this study indicate that it
could occur, which is not surprising given that elk and red deer are subspecies
of Cervus elaphus. The results of this study can be found in the Canadian
Veterinary Journal 51: 169-178.
In addition, it was reported in May 2011 that natural cases of CWD were
found in eight Sika deer (Cervus nippon) and five Sika/red deer crossbreeds
during epidemiological investigations of CWD cases in captive elk in Korea.
May 2011 natural cases of CWD were found in eight Sika deer (Cervus nippon)
and five Sika/red deer crossbreeds during epidemiological investigations of CWD
cases in captive elk in Korea
Friday, May 13, 2011
Chronic Wasting Disease (CWD) outbreaks and surveillance program in the
Republic of Korea Chronic Wasting Disease (CWD) outbreaks and surveillance
program in the Republic of Korea
Hyun-Joo Sohn, Yoon-Hee Lee, Min-jeong Kim, Eun-Im Yun, Hyo-Jin Kim,
Won-Yong Lee, Dong-Seob Tark, In- Soo Cho, Foreign Animal Disease Research
Division, National Veterinary Research and Quarantine Service, Republic of Korea
Chronic wasting disease (CWD) has been recognized as an important prion
disease in native North America deer and Rocky mountain elks. The disease is a
unique member of the transmissible spongiform encephalopathies (TSEs), which
naturally affects only a few species. CWD had been limited to USA and Canada
until 2000. On 28 December 2000, information from the Canadian government showed
that a total of 95 elk had been exported from farms with CWD to Korea. These
consisted of 23 elk in 1994 originating from the so-called “source farm” in
Canada, and 72 elk in 1997, which had been held in pre export quarantine at the
“source farm”.Based on export information of CWD suspected elk from Canada to
Korea, CWD surveillance program was initiated by the Ministry of Agriculture and
Forestry (MAF) in 2001. All elks imported in 1997 were traced back, however elks
imported in 1994 were impossible to identify. CWD control measures included
stamping out of all animals in the affected farm, and thorough cleaning and
disinfection of the premises. In addition, nationwide clinical surveillance of
Korean native cervids, and improved measures to ensure reporting of CWD suspect
cases were implemented. Total of 9 elks were found to be affected. CWD was
designated as a notifiable disease under the Act for Prevention of Livestock
Epidemics in 2002. Additional CWD cases - 12 elks and 2 elks - were diagnosed in
2004 and 2005. Since February of 2005, when slaughtered elks were found to be
positive, all slaughtered cervid for human consumption at abattoirs were
designated as target of the CWD surveillance program. Currently, CWD laboratory
testing is only conducted by National Reference Laboratory on CWD, which is the
Foreign Animal Disease Division (FADD) of National Veterinary Research and
Quarantine Service (NVRQS). In July 2010, one out of 3 elks from Farm 1 which
were slaughtered for the human consumption was confirmed as positive.
Consequently, all cervid – 54 elks, 41 Sika deer and 5 Albino deer – were culled
and one elk was found to be positive. Epidemiological investigations were
conducted by Veterinary Epidemiology Division (VED) of NVRQS in collaboration
with provincial veterinary services. Epidemiologically related farms were found
as 3 farms and all cervid at these farms were culled and subjected to CWD
diagnosis. Three elks and 5 crossbreeds (Red deer and Sika deer) were confirmed
as positive at farm 2. All cervids at Farm 3 and Farm 4 – 15 elks and 47 elks –
were culled and confirmed as negative. Further epidemiological investigations
showed that these CWD outbreaks were linked to the importation of elks from
Canada in 1994 based on circumstantial evidences. In December 2010, one elk was
confirmed as positive at Farm 5. Consequently, all cervid – 3 elks, 11
Manchurian Sika deer and 20 Sika deer – were culled and one Manchurian Sika deer
and seven Sika deer were found to be positive. This is the first report of CWD
in these sub-species of deer. Epidemiological investigations found that the
owner of the Farm 2 in CWD outbreaks in July 2010 had co-owned the Farm 5. In
addition, it was newly revealed that one positive elk was introduced from Farm 6
of Jinju-si Gyeongsang Namdo. All cervid – 19 elks, 15 crossbreed (species
unknown) and 64 Sika deer – of Farm 6 were culled, but all confirmed as
negative. : Corresponding author: Dr. Hyun-Joo Sohn (+82-31-467-1867, E-mail:
shonhj@korea.kr)
2011 Pre-congress Workshop: TSEs in animals and their environment 5
Friday, May 13, 2011
Chronic Wasting Disease (CWD) outbreaks and surveillance program in the
Republic of Korea
Tuesday, June 19, 2012
Experimental Oral Transmission of Chronic Wasting Disease to Reindeer
(Rangifer tarandus tarandus)
I urge everyone to watch this video closely...terry
*** you can see video here and interview with Jeff's Mom, and scientist
telling you to test everything and potential risk factors for humans ***
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 (Aβ) and alphasynuclein (α-syn) aggregates implicated in
Alzheimer's and Parkinson's diseases, respectively. Experiments are ongoing to
study the utility of this technology to detect Aβ and α-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.
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
research article
Stakeholder Perspectives on Chronic Wasting Disease Risk and Management on
the Canadian Prairies
Kari Amick, Douglas Clark & Ryan K. Brook
Page 408-424
Published online: 31 Aug 2015
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Abstract
Chronic wasting disease (CWD) is an infectious disease caused by a prion
that results in neurodegeneration and death in cervids. This study uses Q
methodology to characterize stakeholder perspectives about CWD risk and
management on the Canadian prairies, and to understand the potential for CWD
management using an adaptive governance framework. Workshops and individual
interviews were conducted with 16 stakeholders in Saskatchewan and Manitoba.
Problem definitions framed CWD as a technical problem calling for technical
solutions. All perspectives on solutions focused on the importance of education
and the idea that management should fit within a national management strategy. A
unique Aboriginal perspective also emerged and warrants further exploration.
Results also indicated that although stakeholders wish to be involved with CWD
management, they trust and expect government leadership, and are disinterested
in adaptive governance. Challenges for stakeholder involvement in Canadian CWD
management include a lack of sufficient leadership and general ambivalence.
Keywords: adaptive governance, chronic wasting disease, Q methodology,
stakeholder perspectives, wildlife disease management,
Sunday, September 04, 2016
Stakeholder Perspectives on Chronic Wasting Disease Risk and Management on
the Canadian Prairies
research article
Friday, September 02, 2016
Canada Chronic Wasting Disease CWD Surveillance Update 2016
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!
Monday, August 29, 2016
NWHC USGS CHRONIC WASTING DISEASE CWD TSE PRION UPDATE
Wednesday, August 31, 2016
NORWAY CONFIRMS 4TH CASE OF CHRONIC WASTING DISEASE CWD TSE PRION IN SECOND
CARIBOU
Monday, September 05, 2016
Pathological features of chronic wasting disease in reindeer and
demonstration of horizontal transmission Major Findings for Norway
Tuesday, June 19, 2012
*** Experimental Oral Transmission of Chronic Wasting Disease to Reindeer
(Rangifer tarandus tarandus) ***
Friday, November 22, 2013
Wasting disease is threat to the entire UK deer population
Friday, September 02, 2016
Chronic Wasting Disease Drives Population Decline of White-Tailed
Deer
Thursday, August 25, 2016
FSIS Green Bay Dressed Beef Recalls Beef Products Due To Possible Specified
Risk Materials Contamination the most high risk materials for BSE TSE PRION AKA
MAD COW TYPE DISEASE
Friday, August 26, 2016
Journal Journal of Toxicology and Environmental Health, Part A Volume 79,
2016 - Issue 16-17 Prion Research in Perspective IV CANADA BSE CWD SCRAPIE CJD
TSE Prion Disease
Monday, August 22, 2016
CREUTZFELDT JAKOB DISEASE USA 2015 SPORADIC CJD TOTAL FIGURES REACHES
HIGHEST ANNUAL COUNT TO DATE AT 239 CONFIRMED CASES
Wednesday, December 16, 2015
*** Objects in contact with classical scrapie sheep act as a reservoir for
scrapie transmission ***
*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes
contaminated during neurosurgery ***
Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.
Laboratory of Central Nervous System Studies, National Institute of Neurological
Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.
Stereotactic multicontact electrodes used to probe the cerebral cortex of a
middle aged woman with progressive dementia were previously implicated in the
accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger
patients. The diagnoses of CJD have been confirmed for all three cases. More
than two years after their last use in humans, after three cleanings and
repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were
implanted in the cortex of a chimpanzee. Eighteen months later the animal became
ill with CJD. This finding serves to re-emphasise the potential danger posed by
reuse of instruments contaminated with the agents of spongiform
encephalopathies, even after scrupulous attempts to clean them.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract
Wednesday, September 07, 2016
Michigan Launches an investigation into the Detroit Medical Center dirty,
broken and missing surgical instruments, what about the CJD TSE PRION iatrogenic
threat past and present therefrom?
INCINERATION TEMPS
Requirements include:
a. after burning to the range of 800 to 1000*C to eliminate smell;
well heck, this is just typical public relations fear factor control. do
you actually think they would spend the extra costs for fuel, for such extreme
heat, just to eliminate smell, when they spread manure all over your veg's. i
think not. what they really meant were any _TSE agents_.
b. Gas scrubbing to eliminate smoke -- though steam may be omitted;
c. Stacks to be fitted with grit arreaters;
snip...
1.2 Visual Imact
It is considered that the requirement for any carcase incinerator disign
would be to ensure that the operations relating to the reception, storage and
decepitation of diseased carcasses must not be publicly visible and that any
part of a carcase could not be removed or interfered with by animals or
birds.
full text;
Part II, page 5, final para – continued on page 6.
The precautionary principle, as set out in PPG 23, requires that where
there is perceived to be an unacceptable risk, which cannot be scientifically
quantified, the precautionary principle should be applied. This was the case
with the landfill of untreated carcasses. If there were no perception of such a
risk, as was the case with Thruxted Mill, then the precautionary principle does
not apply. ...
Knacker's yard link to Queniborough nvCJD cluster
Sun, 13 Aug 2000 Jonathan Leake and Dipesh Gadher
Sunday Times Additional reporting: Graham Hind
BRITAIN'S worst outbreak of the human form of mad-cow disease may be linked
to a nearby knacker's yard that sold meat from diseased animals. The yard
operated just eight miles from Queniborough, the Leicestershire village where
health officials are investigating the first known cluster of CJD cases.
Three people who spent time in the village died from CJD in 1998, and a
fourth person is suspected of having the degenerative brain disease. Another
victim lived just three miles away.
The possible link to the knacker's yard - which recycled animals unfit for
human consumption into pet food and other products - dates back 20 years, to
about the time when scientists now believe the BSE epidemic may have
begun.
Two meat traders from Bedfordshire were convicted in 1982 of buying
unapproved beef from W E Mason & Sons of Wigston, near Leicester, and
selling it to an unsuspecting butcher in Hertfordshire.
Last week officials seized council documents and court reports relating to
the company to determine whether any unfit meat may have entered the human food
chain locally.
"We have had a very useful series of conversations about this with Oadby
and Wigston council," said Philip Monk, a consultant in communicable disease
control at Leicestershire health authority, who is heading the Queniborough
investigation. "I am ruling nothing in and nothing out. Anything we have that is
potentially helpful in explaining local meat trading practices has to be
examined."
The case heard by Leicester magistrates in 1982 was the culmination of
Operation Meat Hook, a joint investigation between detectives and environmental
health officers from three counties.
The teams covertly observed Peter Fletcher, a partner in a wholesale
butcher's business near Dunstable, on four occasions in 1980 when he visited
Leonard Mason, the yard's owner. He loaded beef carcasses from the yard into an
un-marked van, which had been contaminated by a cow's head "fouled by stomach
contents", according to evidence given in court. One of the carcasses was later
found to have been infected with pleurisy.
Fletcher marked the meat with a fake inspector's stamp, and then left it
with a retail butcher near Hemel Hempstead, Hertfordshire.
"A knacker's yard may, and frequently will, deal with diseased cattle," the
prosecutor had told an earlier hearing. "Meat may be partly decomposed and
contaminated. Disease is rife in such premises and could include anthrax and
tuberculosis."
Fletcher was jailed for three months and fined ?500. His partner, Francis
Fensome, received a suspended prison sentence. Mason was cleared after telling
the court that he had been told the meat was to be used to feed animals at
Whipsnade zoo [site of two cheetah BSE fatalities -- webmaster]
The knacker's yard, which had been run by the Mason family since 1947, was
closed the same year and now stands derelict. Mason has since died.
Last week his brother, Jack Mason, said: "I am confident there is no
connection with us and the outbreak in Queniborough. Most of the meat went to
zoos. Any meat that was sold locally went to dog owners as pet food."
There is no proof that Mason dealt in cattle infected with BSE, which was
not recognised at the time. But such yards commonly dealt in "downer" cows -
those displaying symptoms of illness - so any animals that did have BSE were
likely to have ended up in such places.
The Queniborough inquiry team is also examining slaughtering techniques at
Leicestershire abattoirs and childhood eating habits of those who grew up in the
village, although school meals have been ruled out as a possible cause of the
CJD outbreak.
Arthur Beyless lost his daughter, Pamela, 24, a bank worker, to the disease
after a two-year struggle for survival. Although the Beylesses live in nearby
Glenfield, Pamela regularly visited her grandparents in Queniborough and the
family often bought meat from Ian Bramley, the village butcher, in the late
1970s and early 1980s.
Beyless said: "On one occasion I was buying some meat when Ian told me he'd
got it for 'a good deal'. It does make you wonder when you consider this theory
about the knacker's yard. This disease is something that might never have
happened if people weren't always grasping for that last penny."
The other two named victims with links to Queniborough are Stacey Robinson,
19, who lived there for 12 years before moving to another part of the county,
and Glen Day, 34, who worked on a farm in the area. He regularly ate at the
Horse and Groom pub, which was supplied with meat by Bramley.
Bramley died in a car crash. His stepmother, Hazel Bramley, said she knew
nothing about Mason's yard. "We bought our meat directly from local farmers,"
she said. "The animals were slaughtered in Leicester and delivered to us. I
don't know anything about this place in Wigston."
18 Jun 00 - CJD - Risk of CJD is higher in north Jonathan Leake
Sunday Times ... Sunday 18 June 2000
Northerners could be at several times more risk from variant CJD , the
human form of "mad cow" disease, than those living in the Midlands and south, a
study by government scientists has found, writes.
The research, carried out by the Creutzfeldt-Jakob disease Surveillance
Unit, also shows that the rate of incidence of the disease, which is always
fatal, is rising across Britain .
The figures remain too low to estimate accurately how many people will
ultimately be affected. Estimates range from hundreds to many thousands .
Variations in the incidence of the disease are a matter of deep concern .
In the north of England - north of Manchester and including Yorkshire and
Humberside - there were 3.14 cases per million people over the five years to
1999. Scotland had the second highest rate at 2.98 cases per million .
The West Midlands emerged as the safest place with just 0.36 cases per
million. East Anglia and the south experienced, respectively, 0.93 and 1.37
cases per
#18 Jun 00 - CJD - Risk of CJD is higher in north
Knacker's yard link to Queniborough nvCJD cluster
Sun, 13 Aug 2000 Jonathan Leake and Dipesh Gadher Sunday Times Additional
reporting: Graham Hind
BRITAIN'S worst outbreak of the human form of mad-cow disease may be linked
to a nearby knacker's yard that sold meat from diseased animals. The yard
operated just eight miles from Queniborough, the Leicestershire village where
health officials are investigating the first known cluster of CJD cases. Three
people who spent time in the village died from CJD in 1998, and a fourth person
is suspected of having the degenerative brain disease. Another victim lived just
three miles away.
The possible link to the knacker's yard - which recycled animals unfit for
human consumption into pet food and other products - dates back 20 years, to
about the time when scientists now believe the BSE epidemic may have
begun.
Two meat traders from Bedfordshire were convicted in 1982 of buying
unapproved beef from W E Mason & Sons of Wigston, near Leicester, and
selling it to an unsuspecting butcher in Hertfordshire.
Last week officials seized council documents and court reports relating to
the company to determine whether any unfit meat may have entered the human food
chain locally.
"We have had a very useful series of conversations about this with Oadby
and Wigston council," said Philip Monk, a consultant in communicable disease
control at Leicestershire health authority, who is heading the Queniborough
investigation. "I am ruling nothing in and nothing out. Anything we have that is
potentially helpful in explaining local meat trading practices has to be
examined."
The case heard by Leicester magistrates in 1982 was the culmination of
Operation Meat Hook, a joint investigation between detectives and environmental
health officers from three counties.
The teams covertly observed Peter Fletcher, a partner in a wholesale
butcher's business near Dunstable, on four occasions in 1980 when he visited
Leonard Mason, the yard's owner. He loaded beef carcasses from the yard into an
un-marked van, which had been contaminated by a cow's head "fouled by stomach
contents", according to evidence given in court. One of the carcasses was later
found to have been infected with pleurisy.
Fletcher marked the meat with a fake inspector's stamp, and then left it
with a retail butcher near Hemel Hempstead, Hertfordshire.
"A knacker's yard may, and frequently will, deal with diseased cattle," the
prosecutor had told an earlier hearing. "Meat may be partly decomposed and
contaminated. Disease is rife in such premises and could include anthrax and
tuberculosis."
Fletcher was jailed for three months and fined ?500. His partner, Francis
Fensome, received a suspended prison sentence. Mason was cleared after telling
the court that he had been told the meat was to be used to feed animals at
Whipsnade zoo [site of two cheetah BSE fatalities -- webmaster]
The knacker's yard, which had been run by the Mason family since 1947, was
closed the same year and now stands derelict. Mason has since died.
Last week his brother, Jack Mason, said: "I am confident there is no
connection with us and the outbreak in Queniborough. Most of the meat went to
zoos. Any meat that was sold locally went to dog owners as pet food."
There is no proof that Mason dealt in cattle infected with BSE, which was
not recognised at the time. But such yards commonly dealt in "downer" cows -
those displaying symptoms of illness - so any animals that did have BSE were
likely to have ended up in such places.
The Queniborough inquiry team is also examining slaughtering techniques at
Leicestershire abattoirs and childhood eating habits of those who grew up in the
village, although school meals have been ruled out as a possible cause of the
CJD outbreak.
Arthur Beyless lost his daughter, Pamela, 24, a bank worker, to the disease
after a two-year struggle for survival. Although the Beylesses live in nearby
Glenfield, Pamela regularly visited her grandparents in Queniborough and the
family often bought meat from Ian Bramley, the village butcher, in the late
1970s and early 1980s.
Beyless said: "On one occasion I was buying some meat when Ian told me he'd
got it for 'a good deal'. It does make you wonder when you consider this theory
about the knacker's yard. This disease is something that might never have
happened if people weren't always grasping for that last penny."
The other two named victims with links to Queniborough are Stacey Robinson,
19, who lived there for 12 years before moving to another part of the county,
and Glen Day, 34, who worked on a farm in the area. He regularly ate at the
Horse and Groom pub, which was supplied with meat by Bramley.
Bramley died in a car crash. His stepmother, Hazel Bramley, said she knew
nothing about Mason's yard. "We bought our meat directly from local farmers,"
she said. "The animals were slaughtered in Leicester and delivered to us. I
don't know anything about this place in Wigston."
Bovine spongiform encephalopathy: Epidemiological studies
The Queniborough CJD cluster
New claims link CJD to water supply
Eurosurveillance, Volume 5, Issue 12, 22 March 2001 Articles
--------------------------------------------------------------------------------
Citation style for this article: Report on Leicestershire vCJD cluster
published. Euro Surveill. 2001;5(12):pii=1785. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1785
Date of submission:
--------------------------------------------------------------------------------
Report on Leicestershire vCJD cluster published
The inquiry team at Leicestershire Health Authority has reported on the
results of the investigation into the geographical cluster of five cases of
variant Creutzfeld-Jakob Disease (vCJD) around the village of Queniborough. The
investigators have concluded that the purchase and consumption of beef in the
early 1980’s from butcher’s shops where the meat could have been contaminated
with brain tissue from cattle affected with bovine spongiform encephalopathy
(BSE) provides a plausible explanation for the cluster (1). A case control
study, in which relatives of the five cases and relatives of 30 age-matched
controls were interviewed, found that cases were 15 times more likely than
controls to have purchased and consumed beef from a butcher who removed brains
from cattle (p = 0.0058, 95% C.I. for odds ratio 1.6 – 140). The two butchers
linked to four of the five cases removed the brains from cattle that were
slaughtered either by the butchers themselves or in a nearby small abattoir.
Pithing rods were used during slaughtering, and the carcasses were cleaned by
wiping rather than by hosing. Removal of the brain was difficult and messy and
the meninges were often ruptured either at removal or by the pithing rod. This
led to a risk of cross contamination of carcass meat with brain tissue. Reasons
are also given as to why during the early 1980’s the cattle in mixed dairy-beef
herds used for the local meat trade may have had higher levels of BSE agent at
slaughter than cattle raised for beef alone.
The practice of removing and selling the brains of cattle as food was legal
in the United Kingdom throughout the 1980’s. Since 1989 it has been illegal for
cattle brains to be used for human consumption and since 1996 the whole head of
cattle over six months must be disposed of in a slaughterhouse as specified risk
material.
The current number of definite and probable cases of vCJD in the UK is 97
(2). Of these, seven are probable cases who are still alive. Although there are
other geographical areas with more than one case, to date Queniborough is the
only area where statistical analysis suggests the association between the cases
is unlikely to have occurred by chance.
References : Bryant G, Monk P. Summary of the final report of the
investigation into the North Leicestershire cluster of variant Creutzfeld-Jakob
disease. Leicester: Leicestershire NHS Health Authority, 2001. Available online
at . Queniborough vCJD cluster report - Department of Health statement [press
release 2001/0141]. London: Department of Health, 21 March 2001. Available
online at
Tue, 8, Aug 2000 19:39:27 -0400
From: jonathan leake
Date: Tue, 8, Aug 2000 19:39:27 -0400
Subject: IN CONFIDENCE (I SMELL A STORY ......)
Sender: jonathan leake
To: BSE Terry Singletary
Message-ID: <200008081939_mc2-af13-1bc compuserve.com=""> MIME-Version:
1.0 Content-Type: text/plain; charset=ISO-8859-1 Content-Disposition: inline
Content-Transfer-Encoding: 8bit X-MIME-Autoconverted: from quoted-printable to
8bit by sys44.hou.wt.net id SAA15659 X-Mozilla-Status: 8007 X-Mozilla-Status2:
00000000 X-UIDL: ed0acd360d74370a3e06000000000000
Hi Terry - this is Jonathan Leake here.
we're thinking of doing a story on the knackers yard meat issue - is there
a link to Queniborough?
Would you mind resending any info you have on this - I may have lost some
of the stuff you sent.
Cd you send it to
jonathan.leake@suandy-times.co.uk
AND TO
dipesh.gadher@sunday-times.co.uk
- HE'S RESEARCHING THIS STORY FOR ME AS I'M AT A CONFERENCE
MANY THANKS FOR YOUR HELP - AND FOR ALL THE GOOD WORK YOU'VE BEEN
DOING
snip...end...TSS
=========================================================
Re: IN CONFIDENCE (I SMELL A STORY )
Subject: Re: IN CONFIDENCE (I SMELL A STORY )
Date: Tue, 08 Aug 2000 21:41:57 -0700
From: "Terry S. Singeltary Sr."
To: jonathan leake
Hello Jonathan,
yes, give me some time though. there is a shitstorm on CJD Voice, they let
the Faillace's on the CJD Voice support group (TSE tainted sheep farmers)
without telling anyone; and myself and other are pissed off to say the least.
This was suppose to be a support group. i told them it would be like asking the
Malboro Man on a Cancer List. But he is Dead. Maybe it struck a nerve.
Have you got the DFA 4, 5, and 7, i thought i read something about knackers
or maybe babyfoods??? not sure. i can send to you. I am sure i have something in
the GBR's for the states and the other countries, don't have time to read. you
can read them at;
i will search as soon as i get time ....
kind regards, Terry
jonathan leake wrote:
Hi Terry - this is Jonathan Leake here. we're thinking of doing a story on
the knackers yard meat issue - is there a link to Queniborough?
Would you mind resending any info you have on this - I may have lost some
of the stuff you sent. ...
snip...END...TSS
Re: KNACKERS AND RENDERS
Subject: Re: KNACKERS AND RENDERS
Date: Thu, 10 Aug 2000 16:04:14 ·0700
From: "Terry S. Singeltary Sr."
To: jonathan.leake@sunday-times.co.uk,
dipesh.gadher@Sunday-times.co.uk
do you have access to the;
The Veterinary-Record, December 20/27, 1997 Papers and Articles Effect of
rendering procedures on the scrapie agent D. M. Taylor, S.L. Woodgate, A.J.
Fleetwood, R.J.G. Cawthorne it's about 6 or 7 pages. i do not have it scanned
and it's fairly fine print, however good print. also the report; The Veterinary
Record, March 2, 1991 Papers and Articles Bovine Spongiform Encephalopathy:
epidemiological studies on the origin there is a good section of rendering;
Survey of rendering processes, solvents etc (very detailed on temps and
processes) can scan copy correct and paste, but it will take some time, or fax
COLLECT to you. I'm running out of quarters fast, nobody paying me to do this,
and i am on disability. so the fax will have to be collect ... regards, Terry 1
of 1 8/13/00 1 :06 PM
end...TSS
Date: Fri, 2 Mar 2001 23:27:10 +0000 (GMT)
From:
Subject: confidential
To: "Terry S. Singeltary Sr."
Okay, you need to know. You don't need to pass it on as nothing will come
of it and there is not a damned thing anyone can do about it. Don't even hint at
it as it will be denied and laughed at..........
USDA is gonna do as little as possible until there is actually a human case
in the USA of the nvcjd........
if you want to move this thing along and shake the earth....then we gotta
get the victims families to make sure whoever is doing the autopsy is credible,
trustworthy, and a saint with the courage of Joan of Arc........
I am not kidding!!!! so, unless we get a human death from EXACTLY the same
form with EXACTLY the same histopath lesions as seen in the UK
nvcjd........
forget any action........it is ALL gonna be sporadic!!!
And, if there is a case.......there is gonna be every effort to link it to
international travel, international food, etc. etc. etc. etc. etc. They will go
so far as to find out if a sex partner had ever traveled to the UK/europe, etc.
etc. ....
It is gonna be a long, lonely, dangerous twisted journey to the truth. They
have all the cards, all the money, and are willing to threaten and carry out
those threats....
and this may be their biggest downfall.
=========================================
snip...
Evidence That Transmissible Mink Encephalopathy Results from Feeding
Infected Cattle
Over the next 8-10 weeks, approximately 40% of all the adult mink on the
farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or
dead dairy cattle...
snip...
for anyone interested, see full text ;
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
Terry S. Singeltary Sr.
