Article Citation: Lisa L. Wolfe, Karen A. Fox, and Michael W. Miller (2014)
“Atypical” Chronic Wasting Disease in PRNP Genotype 225FF Mule Deer.
Journal of Wildlife Diseases In-Press. doi: http://dx.doi.org/10.7589/2013-10-274
Ahead of Print “Atypical” Chronic Wasting Disease in PRNP Genotype 225FF
Mule Deer Lisa L. Wolfe 1,2, Karen A. Fox 1, and Michael W. Miller 1 1 Colorado
Division of Parks and Wildlife, Wildlife Research Center, 317 West Prospect
Road, Fort Collins, Colorado 80526-2097, USA
Abstract
We compared mule deer (Odocoileus hemionus) of two different PRNP genotypes
(225SS, 225FF) for susceptibility to chronic wasting disease (CWD) in the face
of environmental exposure to infectivity. All three 225SS deer had
immunohistochemistry (IHC)-positive tonsil biopsies by 710 days postexposure
(dpe), developed classic clinical signs by 723–1,200 dpe, and showed gross and
microscopic pathology, enzyme-linked immunosorbent assay (ELISA) results, and
IHC staining typical of prion disease in mule deer. In contrast, although all
three 225FF deer also became infected, the two individuals surviving >720 dpe
had consistently negative biopsies, developed more-subtle clinical signs of CWD,
and died 924 or 1,783 dpe. The 225FF deer were “suspect” by ELISA postmortem but
showed negative or equivocal IHC staining of lymphoid tissues; both clinically
affected 225FF deer had spongiform encephalopathy in the absence of IHC staining
in the brain tissue. The experimental cases resembled three cases encountered
among five additional captive 225FF deer that were not part of our experiment
but also died from CWD. Aside from differences in clinical disease presentation
and detection, 225FF mule deer also showed other, more-subtle, atypical traits
that may help to explain the rarity of this genotype in natural populations,
even in the presence of enzootic CWD.
Key words: Chronic wasting disease, genotype, mule deer, Odocoileus
hemionus, prion, PRNP
Received: October 21, 2013; Accepted: January 22, 2014 ;Published Online:
May 7, 2014
2 Corresponding author (email: lisa.wolfe@state.co.us)
DEPARTMENT OF HEALTH & HUMAN SERVICES PUBLIC HEALTH SERVICE FOOD AND
DRUG ADMINISTRATION
April 9, 2001 WARNING LETTER
01-PHI-12 CERTIFIED MAIL RETURN RECEIPT REQUESTED
Brian J. Raymond, Owner Sandy Lake Mills 26 Mill Street P.O. Box 117 Sandy
Lake, PA 16145 PHILADELPHIA DISTRICT
Tel: 215-597-4390
Dear Mr. Raymond:
Food and Drug Administration Investigator Gregory E. Beichner conducted an
inspection of your animal feed manufacturing operation, located in Sandy Lake,
Pennsylvania, on March 23, 2001, and determined that your firm manufactures
animal feeds including feeds containing prohibited materials. The inspection
found significant deviations from the requirements set forth in Title 21, code
of Federal Regulations, part 589.2000 - Animal Proteins Prohibited in Ruminant
Feed. The regulation is intended to prevent the establishment and amplification
of Bovine Spongiform Encephalopathy (BSE) . Such deviations cause products being
manufactured at this facility to be misbranded within the meaning of Section
403(f), of the Federal Food, Drug, and Cosmetic Act (the Act).
Our investigation found failure to label your swine feed with the required
cautionary statement "Do Not Feed to cattle or other Ruminants" The FDA suggests
that the statement be distinguished by different type-size or color or other
means of highlighting the statement so that it is easily noticed by a purchaser.
In addition, we note that you are using approximately 140 pounds of cracked
corn to flush your mixer used in the manufacture of animal feeds containing
prohibited material. This flushed material is fed to wild game including deer, a
ruminant animal. Feed material which may potentially contain prohibited material
should not be fed to ruminant animals which may become part of the food chain.
The above is not intended to be an all-inclusive list of deviations from
the regulations. As a manufacturer of materials intended for animal feed use,
you are responsible for assuring that your overall operation and the products
you manufacture and distribute are in compliance with the law. We have enclosed
a copy of FDA's Small Entity Compliance Guide to assist you with complying with
the regulation... blah, blah, blah...
PRODUCT
Product is __custom made deer feed__ packaged in 100 lb. poly bags. The
product has no labeling. Recall # V-003-5.
CODE
The product has no lot code. All custom made feed purchased between June
24, 2004 and September 8, 2004.
RECALLING FIRM/MANUFACTURER
Farmers Elevator Co, Houston, OH, by telephone and letter dated September
27, 2004. Firm initiated recall is ongoing.
REASON
Feed may contain protein derived from mammalian tissues which is prohibited
in ruminant feed.
VOLUME OF PRODUCT IN COMMERCE
Approximately 6 tons.
DISTRIBUTION OH.
END OF ENFORCEMENT REPORT FOR October 20, 2004
################# BSE-L-subscribe-request@uni-karlsruhe.de
#################
DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer
and Elk in Animal Feed; Availability
Date: Fri, 16 May 2003 11:47:37 –0500
EMC 1 Terry S. Singeltary Sr. Vol #: 1
Oral transmission and early lymphoid tropism of chronic wasting disease
PrPres in mule deer fawns (Odocoileus hemionus )
snip...
These results indicate that CWD PrP res can be detected in lymphoid tissues
draining the alimentary tract within a few weeks after oral exposure to
infectious prions and may reflect the initial pathway of CWD infection in deer.
The rapid infection of deer fawns following exposure by the most plausible
natural route is consistent with the efficient horizontal transmission of CWD in
nature and enables accelerated studies of transmission and pathogenesis in the
native species.
snip...
*** These findings support oral exposure as a natural route of CWD
infection in deer and support oral inoculation as a reasonable exposure route
for experimental studies of CWD.
snip...
PLEASE SEE FULL TEXT SUBMISSION ;
Sunday, December 15, 2013
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
http://madcowusda.blogspot.com/2013/12/fda-part-589-substances-prohibited-from.html
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.
***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.
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.
Saturday, March 15, 2014
Potential role of soil properties in the spread of CWD in western Canada
The routes of CWD transmission remain unclear. CWD is a contagious prion
diease, the infectious agent is released in various body fluids including
saliva, feces, blood and urine.4 Although the majority of studies suggest an
oral route of exposure to be responsible for environmental transmission,5 there
is also evidence for intranasal and aerosol transmission6,7 as contributing
factors. In all transmission routes, soils can serve as a stable reservoir of
prion diseases (transmissible spongiform encephalopathies, TSEs). Prions bound
to soil particles Can remain infectious in the soils for many years.8,9
Therefore, soil properties are an important factor for PrPTSE preservation and
transmission in the environment.10-13 Analysis of soil-prion interactions and
the impact on infectivity is a complicated task because soils are multicomponent
systems consisting of mineral particles (clay. silt, sand); soil organic matter
(humic, fulvic acids and humin); humus or/and Fe-Mn film and cutans interacting
with mineral particles. The enormous complexity of soils indicates a need to
examine a variety of soils and their separated compounds (mineral and organic)
to identify the ability of prions to bind the soil, what the effect of binding
is on infectivity and what components of soil bind prions. ...
Friday, February 08, 2013
*** Behavior of Prions in the Environment: Implications for Prion Biology
Monday, January 05, 2009
CWD, GAME FARMS, BAITING, AND POLITICS
Elk and Deer Use of Mineral Licks: Implications for Disease Transmission
Results from the mineral analyses combined with camera data revealed that
visitation was highest at sodium-rich mineral licks. Mineral licks may play a
role in disease transmission by acting as sites of increased interaction as well
as reservoirs for deposition, accumulation, and ingestion of disease agents.
Friday, October 26, 2012
CHRONIC WASTING DISEASE CWD PENNSYLVANIA GAME FARMS, URINE ATTRACTANT
PRODUCTS, BAITING, AND MINERAL LICKS
Sunday, September 01, 2013 hunting over gut piles and CWD TSE prion disease
New studies on the heat resistance of hamster-adapted scrapie agent:
Threshold survival after ashing at 600°C suggests an inorganic template of
replication
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel
Production
Detection of protease-resistant cervid prion protein in water from a
CWD-endemic area
A Quantitative Assessment of the Amount of Prion Diverted to Category 1
Materials and Wastewater During Processing
Rapid assessment of bovine spongiform encephalopathy prion inactivation by
heat treatment in yellow grease produced in the industrial manufacturing process
of meat and bone meals
PPo4-4:
Survival and Limited Spread of TSE Infectivity after Burial
Monday, May 05, 2014
Member Country details for listing OIE CWD 2013 against the criteria of
Article 1.2.2., the Code Commission recommends consideration for listing
Program Standards: Chronic Wasting Disease Herd Certification Program and
Interstate Movement of Farmed or Captive Deer, Elk, and Moose
DOCUMENT ID: APHIS-2006-0118-0411
***Singeltary submission
*** 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.
The chances of a person or domestic animal contracting CWD are “extremely
remote,” Richards said. The possibility can’t be ruled out, however. “One could
look at it like a game of chance,” he explained. “The odds (of infection)
increase over time because of repeated exposure. That’s one of the downsides of
having CWD in free-ranging herds: ***We’ve got this infectious agent out there
that we can never say never to in terms of (infecting) people and domestic
livestock.”
P35
*** 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.
*** 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).
Saturday, April 19, 2014
Exploring the zoonotic potential of animal prion diseases: In vivo and in
vitro approaches
Sunday, May 18, 2014
*** Chronic Wasting Disease CWD TSE PRION DISEASE and the transmission to
other species
Thursday, January 2, 2014
*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant
Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***
Wednesday, December 11, 2013
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic
Creutzfeldt-Jakob Disease ***
Sunday, April 06, 2014
SPORADIC CJD and the potential for zoonotic transmission there from, either
directly or indirectly via friendly fire iatrogenic mode, evidence to date
Friday, January 10, 2014
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type
prion disease, what it ???
Monday, May 19, 2014
Variant CJD: 18 years of research and surveillance
TSS
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