NJ S2024 - Establishes licensing program in Department of Agriculture for
farmed deer and other cervids.
Introduced Chamber:upper
Introduced Session:2012-2013 Regular Session
Bill Summary:
This bill would designate farmed cervids (deer and similar hoofed mammals)
that are part of a Cervidae livestock operation as agricultural livestock, and
establish, in the Department of Agriculture, a licensing program for Cervidae
livestock operations. Under the bill, the Department of Agriculture would adopt
regulations for the operation of Cervidae livestock operations. In 2002, the
Department of Environmental Protection's (DEP) Division of Fish and Wildlife
banned the importation of deer and elk into the State as a precautionary measure
to prevent the spread of chronic wasting disease into the State's wild and
farmed herds of animals belonging to the deer family. Under current rules, a
person wishing to import cervids into the State must comply with requirements
set forth by both the Department of Agriculture and the DEP's Division of Fish
and Wildlife. This bill would establish the authority for the regulation of
farmed cervid herds in the Department of Agriculture. This bill is similar to
legislation, HB 1580, enacted in Pennsylvania in 2006, which provides that
Cervidae livestock operations are to be considered normal agricultural
operations and gives the responsibility for regulating these operations to the
Pennsylvania Department of Agriculture. This bill also provides that the DEP and
the Fish and Game Council would have no authority to promulgate rules or
regulations concerning Cervidae livestock operations. However, the DEP and the
Fish and Game Council may regulate the possession of cervids that are not part
of a Cervidae livestock operation as defined by the bill. In addition, the bill
would not exempt any person from fish and wildlife laws, or any rules or
regulations adopted pursuant thereto, concerning the release or escape of farmed
cervids into the wild.
Bill Subjects:
Subjects were not specified
Sponsors:
Jeff Van Drew (D), Jeff Van Drew (D)
5/31/2012 Introduced in the Senate, Referred to Senate Economic Growth
Committee
ASSEMBLY, No. 4091 STATE OF NEW JERSEY 214th LEGISLATURE INTRODUCED JUNE
13, 2011
b. exempt any person from the provisions of Title 23 of the
31 Revised Statutes, or any rules or regulations adopted pursuant thereto,
concerning the release or escape of farmed cervids into the wild.
Greetings New Jersey Hunters et al,
Please think long and hard before approving this bill.
how many states have $465,000., and can quarantine and purchase there from,
each cwd said infected farm, but how many states can afford this for all the cwd
infected cervid game ranch type farms ???
? game farms in a state X $465,000., do all these game farms have insurance
to pay for this risk of infected the wild cervid herds, in each state ???
USDA-APHIS-VS Chronic Wasting Disease National Program
Patrice N. Klein of USDA APHIS VS – National Center for Animal Health
Programs provided an update on the agency’s CWD–related activities:
CWD Rule Update: The amended final rule on chronic wasting disease (CWD) is
currently in departmental clearance. The rule will set minimum standards for
interstate movement and establish the national voluntary Herd Certification
Program (HCP). Farmed/captive cervid surveillance testing: Through FY2010, VS
conducted surveillance testing on approximately 20,000 farmed /captive cervids
by the immunohistochemistry (IHC) standard protocol. As of September 15, 2011,
approximately 19,000 farmed /captive cervids were tested by IHC for CWD with
funding to cover lab costs provided through NVSL.
Farmed/captive cervid CWD status: The CWD positive captive white-tailed
deer (WTD) herd reported in Missouri (February 2010) was indemnified and
depopulation activities were completed in June 2011. All depopulated animals
were tested for CWD and no additional CWD positive animals were found.
In FY 2011, CWD was reported in two captive elk herds in Nebraska
(December, 2010 and April 2011, respectively).
To date, 52 farmed/captive cervid herds have been identified in 11 states:
CO, KS, MI, MN, MO, MT, NE, NY, OK, SD, WI.
Thirty-nine were elk herds and 13 were WTD herds. At this time, eight CWD
positive herds remain – six elk herds in Colorado and the two elk herds in
Nebraska.
Wild Cervid surveillance: In FY 2009 funding supported surveillance in
approximately 74,330 wild cervids in 47 cooperating States. Wild cervid CWD
surveillance totals are pending for fiscal year 2010 (2010 – 2011 calendar year)
due to seasonal surveillance activities and completion of final cooperative
agreement reporting to APHIS.
In fiscal year 2011, there are 15 ‘tier 1’ States, 20 ‘tier 2’ States, and
15 ‘tier 3’ States. Two new ‘tier 1’ States, Minnesota and Maryland, were added
in fiscal year 2011 based on the new CWD detections in a free-ranging
white-tailed deer in southeastern Minnesota and in western Maryland.
Consequently, Delaware was upgraded to ‘tier 2’ status as an adjacent State to
Maryland. For FY 2011, 45 States and 32 Tribes will receive cooperative
agreement funds to complete wild cervid surveillance and other approved work
plan activities. Based on FY 2012 projected budget reductions, future
cooperative agreement funds will be eliminated.
APHIS CWD Funding: In FY2011, APHIS received approximately $15.8 million in
appropriated funding for the CWD Program. The President’s FY 2012 budget
proposes to reduce program funding for CWD by $13.9 million, leaving the program
with a request of $1.925 million to provide some level of Federal coordination
for the national herd certification program (HCP).
Consequently, APHIS is planning to amend its role in the program to one of
Federal coordination. Based on the projected FY 2012 budget, funding for CWD
cooperative agreements and indemnity funding for States and Tribes will be
eliminated. Under this scenario, the States or cervid industry producers will
likely be responsible for the costs of surveillance testing and indemnity for
appraisal, depopulation, and disposal of CWD-positive animals.
Commodity Health Line Structure: In the FY 2012 budget, livestock
commodities regulated by USDA have been organized into ‘Commodity Health Line’
structures or groupings. APHIS’ Equine, Cervid and Small Ruminant (ECSR) Health
line supports efforts to protect the health and thereby improve the quality and
productivity of the equine, cervid and small ruminant industries. Activities
supported by the ECSR Health line range from monitoring and surveillance to
investigation and response actions undertaken when health issues relevant to the
industry are identified. APHIS also maintains regulations and program standards
which guide ECSR activities at both the Federal and State/Tribal level.
The ECSR Health line funds essential activities necessary to maintain
current ECSR surveillance and program operations while providing the flexibility
to respond to new and emerging industry-specific health concerns. APHIS’ current
activities include Scrapie, Chronic Wasting Disease (CWD), Slaughter Horse
Transport, and Brucellosis/Tuberculosis in cervids. Overall, APHIS will use
funding from the ECSR Health Line Item to support Agency efforts in the
following mission areas: prevention, preparedness and communication; monitoring,
surveillance and detection; response and containment; and continuity of
business, mitigation and recovery
Scrapie in Deer: Comparisons and Contrasts to Chronic Wasting Disease (CWD)
Justin J. Greenlee of the Virus and Prion Diseases Research Unit, National
Animal Disease Center, ARS, USDA, Ames, IA provided a presentation on scrapie
and CWD in inoculated deer. Interspecies transmission studies afford the
opportunity to better understand the potential host range and origins of prion
diseases. We inoculated white-tailed deer intracranially (IC) and by a natural
route of exposure (concurrent oral and intranasal inoculation) with a US scrapie
isolate. All deer inoculated by the intracranial route had evidence of PrPSc
accumulation and those necropsied after 20 months post-inoculation (PI) (3/5)
had clinical signs, spongiform encephalopathy, and widespread distribution of
PrPSc in neural and lymphoid tissues. A single deer that was necropsied at 15.6
months PI did not have clinical signs, but had widespread distribution of PrPSc.
This highlights the facts that 1) prior to the onset of clinical signs PrPSc is
widely distributed in the CNS and lymphoid tissues and 2) currently used
diagnostic methods are sufficient to detect PrPSc prior to the onset of clinical
signs. The results of this study suggest that there are many similarities in the
manifestation of CWD and scrapie in white-tailed deer after IC inoculation
including early and widespread presence of PrPSc in lymphoid tissues, clinical
signs of depression and weight loss progressing to wasting, and an incubation
time of 21-23 months. Moreover, western blots (WB) done on brain material from
the obex region have a molecular profile consistent with CWD and distinct from
tissues of the cerebrum or the scrapie inoculum. However, results of microscopic
and IHC examination indicate that there are differences between the lesions
expected in CWD and those that occur in deer with scrapie: amyloid plaques were
not noted in any sections of brain examined from these deer and the pattern of
immunoreactivity by IHC was diffuse rather than plaque-like. After a natural
route of exposure, 100% of white-tailed deer were susceptible to scrapie. Deer
developed clinical signs of wasting and mental depression and were necropsied
from 28 to 33 months PI. Tissues from these deer were positive for scrapie by
IHC and WB. Tissues with PrPSc immunoreactivity included brain, tonsil,
retropharyngeal and mesenteric lymph nodes, hemal node, Peyer’s patches, and
spleen. While two WB patterns have been detected in brain regions of deer
inoculated by the natural route, unlike the IC inoculated deer, the pattern
similar to the scrapie inoculum predominates.
Committee Business:
The Committee discussed and approved three resolutions regarding CWD. They
can be found in the report of the Reswolutions Committee. Essentially the
resolutions urged USDA-APHIS-VS to:
Continue to provide funding for CWD testing of captive cervids
Finalize and publish the national CWD rule for Herd Certification and
Interstate Movement
Evaluate live animal test, including rectal mucosal biopsy, for CWD in
cervids
how many states have $465,000., and can quarantine and purchase there from,
each cwd said infected farm, but how many states can afford this for all the cwd
infected cervid game ranch type farms ???
? game farms in a state X $465,000., do all these game farms have insurance
to pay for this risk of infected the wild cervid herds, in each state ???
Tuesday, December 20, 2011
CHRONIC WASTING DISEASE CWD WISCONSIN Almond Deer (Buckhorn Flats) Farm
Update DECEMBER 2011
The CWD infection rate was nearly 80%, the highest ever in a North American
captive herd.
RECOMMENDATION: That the Board approve the purchase of 80 acres of land for
$465,000 for the Statewide Wildlife Habitat Program in Portage County and
approve the restrictions on public use of the site.
Form 1100-001
(R 2/11)
NATURAL RESOURCES BOARD AGENDA ITEM
SUBJECT: Information Item: Almond Deer Farm Update
FOR: DECEMBER 2011 BOARD MEETING
TUESDAY
TO BE PRESENTED BY TITLE: Tami Ryan, Wildlife Health Section Chief
SUMMARY:
SNIP...
2010 WISCONSIN CAPTIVE DEER ESCAPES
There were 26 reported escape incidents so far this year, this amounted to
20 actual confirmed escape incidents because 3 were previously reported, 2 were
confirmed as wild deer, and 1 incident was not confirmed. ...
snip...
Deer, elk continue to escape from state farms
Article by: DOUG SMITH , Star Tribune Updated: March 14, 2011 - 12:08 PM
Curbing chronic wasting disease remains a concern; officials are increasing
enforcement.
Almost 500 captive deer and elk have escaped from Minnesota farms over the
past five years, and 134 were never recaptured or killed.
So far this year, 17 deer have escaped, and officials are still searching
for many of those.
see ;
Thursday, October 11, 2012
Pennsylvania Confirms First Case CWD Adams County Captive Deer Tests
Positive
Pennsylvania CWD number of deer exposed and farms there from much greater
than first thought
Published: Wednesday, October 17, 2012, 10:44 PM Updated: Wednesday,
October 17, 2012, 11:33 PM
Tuesday, October 23, 2012
PA Captive deer from CWD-positive farm roaming free
Wednesday, November 07, 2012
PENNSYLVANIA Second Adams County Deer Tests Positive for Chronic Wasting
Disease
Friday, September 28, 2012
Stray elk renews concerns about deer farm security Minnesota
Monday, June 11, 2012
OHIO Captive deer escapees and non-reporting
INDIANA 20 DEER ESCAPE TROPHY BUCK GAME FARM STATE OFFICIALS FEAR CWD RISK
TO WILD
Friday, July 20, 2012
CWD found for first time in Iowa at hunting preserve
Wednesday, September 05, 2012
Additional Facility in Pottawatamie County Iowa Under Quarantine for CWD
after 5 deer test positive
Friday, September 21, 2012
Chronic Wasting Disease CWD raises concerns about deer farms in Iowa
MORE CERVIDS ESCAPE GAME FARMS AND POTENTIALLY EXPOSE THE WILD HERD TO CWD
AND OTHER DANGEROUS PATHOGENS
Two ‘elk’ slain near Antoich were European red deer that escaped from farm
BY DALE BOWMAN For Sun-Times Media November 8, 2012 10:28PM
Updated: November 9, 2012 2:31AM
It’s mistaken identity gone wild. Ron Mulholland thought he arrowed two
wild elk last Friday from his deer stand on a farm outside of Antioch.
When James Minogue saw the story in Wednesday’s Sun-Times, he recognized
the pair of breeding European red deer from the herd he helps manage for Avery
Brabender on a farm in unincorporated Antioch. They, along with four others,
escaped some time after Oct. 31 when a gate was opened or left open.
“It amazed me that they think they are elk and wild,’’ Minogue said.
However, elk and red deer are close enough to interbreed.
“I will talk to him,’’ Mulholland said. “I assumed they were wild and
killed them. To me, they were elk. I don’t know. ... I feel bad for the guy that
he would lose them. I reacted because I assumed it was an elk and I shot
him.’’
“You don’t see elk in the wild in Illinois,’’ said Kevin Bettis, the duty
officer in Springfield Thursday for the Illinois Conservation Police.
That’s tricky. A decade ago, Illinois didn’t have wolves or cougars,
either. Both species now make regular appearances.
“These animals were hand-fed: We feed them bread, apples, corn,,’’ Minogue
said. Another tricky part is neither elk nor European red deer are protected or
regulated under Illinois’ wildlife code. But these European red deer are
considered domesticated animals. The herd is registered with the Illinois
Department of Agriculture.
“It is no different than shooting a cow,’’ Bettis said.
However, Capt. Neal Serdar of Region II (northeast Illinois) checked with
CPOs in southern Illinois, where escaped animals of such sort are more a more
frequent issue.
Then he said, “The individual who shot the two red deer did not break any
laws.’’
The Illinois Conservation Police consider the case closed. Whether there is
any civil case would seem tricky at best, since the animals were loose.
Minogue said they recaptured two of the red deer already. He said the
reason there were no ear tags is because they are a “contained, monitored
herd.’’
It sounds like both parties can work it out.
“If it gets down to that, I would give him the antlers,’’ Mulholland said.
“But I kind of feel it is his responsibility.’’
Thursday, February 09, 2012
50 GAME FARMS IN USA INFECTED WITH CHRONIC WASTING DISEASE
Friday, February 03, 2012
Wisconsin Farm-Raised Deer Farms and CWD there from 2012 report Singeltary
et al
CWD, GAME FARMS, BAITING, AND POLITICS
Friday, August 31, 2012
COMMITTEE ON CAPTIVE WILDLIFE AND ALTERNATIVE LIVESTOCK and CWD 2009-2012 a
review
Tuesday, June 05, 2012
Captive Deer Breeding Legislation Overwhelmingly Defeated During 2012
Legislative Session
Friday, August 24, 2012
Diagnostic accuracy of rectal mucosa biopsy testing for chronic wasting
disease within white-tailed deer (Odocoileus virginianus) herds in North America
The overall diagnostic specificity was 99.8%. Selective use of antemortem
rectal biopsy sample testing would provide valuable information during disease
investigations of CWD-suspect deer herds.
*** Friday, October 12, 2012
Texas Animal Health Commission (TAHC) is Now Accepting Comments on Rule
Proposals for “Chronic Wasting Disease (CWD)”
TO: comments@tahc.state.tx.us; Texas Animal Health Commission (TAHC)
In Chronic Wasting disease (CWD) of deer several careful studies have been
performed that, together with our present finding, depose in favor of airborne
transmission in this naturally occurring disease. Indeed, CWD prions can be
transmitted experimentally via aerosol and the nasal route to transgenic
cervidized mice.33 Although no anecdotal or epidemiological evidence has come
forward that airborne transmission may be important for the spread of CWD,
several lines of thought suggest that this possibility is not implausible. In
deer, prions have been detected in urine, saliva, feces and blood of diseased
animals. Moreover, it was claimed that pathological prion protein could be
recovered from the environmental water in an endemic area.34 Since all fluids
can act as sources for the generation of aerosols, any of the body fluids
mentioned above may represent the point of origin for airborne transmission of
CWD prions. In this context, also the presence of infectious prions in blood of
patients should be mentioned which was demonstrated by the transmission of vCJD
by blood transfusions.35,36 The growing body of evidence that prion transmission
can be airborne—at least under certain conditions—dictates that the release of
potentially contaminated aerosols should be avoided under all circumstances.
snip...
In conclusion, aerosols can infect mice with a surprisingly high
efficiency. Just how important a role is played by this newly recognized pathway
of spread in natural transmission is, as of now, unclear and in need of further
studies. Although it was not identified as a route of infection in
epidemiological studies thus far, the worryingly high attack rate suggests that
we would be well-advised to carefully avoid the inhalation of aerosols from
prion-containing materials. Key words: prion, prion transmission, scrapie,
chronic wasting diseases, CWD, Creutzfeldt-Jacob-disease, CJD, TSE, aerosol,
pathogens, allergens Submitted: 05/19/11 Accepted: 06/09/11 DOI:
10.4161/pri.5.3.16851 *Correspondence to: Lothar Stitz or Adriano Aguzzi; Email:
lothar.stitz@fli.bund.de or adriano.aguzzi@usz.ch
snip...see full text ;
PLEASE SEE FULL TEXT, AND AGAIN, many thanks to PLOS for open access !!!
Monday, September 17, 2012
Rapid Transepithelial Transport of Prions Following Inhalation
Thursday, December 29, 2011
Aerosols An underestimated vehicle for transmission of prion diseases?
PRION www.landesbioscience.com
please see more on Aerosols and TSE prion disease here ;
CWD has been identified in free-ranging cervids in 15 US states and 2
Canadian provinces and in ≈ 100 captive herds in 15 states and provinces and in
South Korea (Figure 1, panel B). SNIP... Long-term effects of CWD on cervid
populations and ecosystems remain unclear as the disease continues to spread and
prevalence increases. In captive herds, CWD might persist at high levels and
lead to complete herd destruction in the absence of human culling. Epidemiologic
modeling suggests the disease could have severe effects on free-ranging deer
populations, depending on hunting policies and environmental persistence (8,9).
CWD has been associated with large decreases in free-ranging mule deer
populations in an area of high CWD prevalence (Boulder, Colorado, USA) (5).
PLEASE STUDY THIS MAP, COMPARE FARMED CWD TO WILD CWD...TSS
Saturday, February 18, 2012
Occurrence, Transmission, and Zoonotic Potential of Chronic Wasting Disease
CDC Volume 18, Number 3—March 2012
CWD has been identified in free-ranging cervids in 15 US states and 2
Canadian provinces and in ≈100 captive herds in 15 states and provinces and in
South Korea (Figure 1, panel B).
Research Paper
Salivary prions in sheep and deer
Volume 6, Issue 1 January/February/March 2012
Pages 52 – 61
Gültekin Tamgüney, Jürgen A. Richt, Amir N. Hamir, Justin J. Greenlee,
Michael W. Miller, Lisa L. Wolfe, Tracey M. Sirochman, Alan J. Young, David V.
Glidden, Natrina L. Johnson, Kurt Giles, Stephen J. DeArmond and Stanley B.
Prusiner
Gültekin Tamgüney Institute for Neurodegenerative Diseases; Department of
Neurology; University of California, San Francisco, CA USA Jürgen A. Richt
National Animal Disease Center, ARS-USDA; Ames, IA USA Amir N. Hamir National
Animal Disease Center, ARS-USDA; Ames, IA USA Justin J. Greenlee National Animal
Disease Center, ARS-USDA; Ames, IA USA Michael W. Miller Colorado Division of
Wildlife, Wildlife Research Center; Fort Collins, CO USA Lisa L. Wolfe Colorado
Division of Wildlife, Wildlife Research Center; Fort Collins, CO USA Tracey M.
Sirochman Colorado Division of Wildlife, Wildlife Research Center; Fort Collins,
CO USA Alan J. Young Department of Veterinary Science, South Dakota State
University; Brookings, SD USA David V. Glidden Departments of Epidemiology and
Biostatistics; University of California, San Francisco, CA USA Natrina L.
Johnson Institute for Neurodegenerative Diseases; San Francisco, CA USA Kurt
Giles Institute for Neurodegenerative Diseases; Department of Neurology;
University of California, San Francisco, CA USA Stephen J. DeArmond Institute
for Neurodegenerative Diseases; San Francisco, CA USA; Department of Pathology,
University of California; San Francisco, CA USA Stanley B. Prusiner
Corresponding author: stanley@ind.ucsf.edu Institute for Neurodegenerative
Diseases; Department of Neurology; University of California, San Francisco, CA
USA
Scrapie of sheep and chronic wasting disease (CWD) of cervids are
transmissible prion diseases. Milk and placenta have been identified as sources
of scrapie prions but do not explain horizontal transmission. In contrast, CWD
prions have been reported in saliva, urine and feces, which are thought to be
responsible for horizontal transmission. While the titers of CWD prions have
been measured in feces, levels in saliva or urine are unknown. Because sheep
produce ~17 L/day of saliva, and scrapie prions are present in tongue and
salivary glands of infected sheep, we asked if scrapie prions are shed in
saliva. We inoculated transgenic (Tg) mice expressing ovine prion protein,
Tg(OvPrP) mice, with saliva from seven Cheviot sheep with scrapie. Six of seven
samples transmitted prions to Tg(OvPrP) mice with titers of -0.5 to 1.7 log ID50
U/ml. Similarly, inoculation of saliva samples from two mule deer with CWD
transmitted prions to Tg(ElkPrP) mice with titers of -1.1 to -0.4 log ID50 U/ml.
Assuming similar shedding kinetics for salivary prions as those for fecal prions
of deer, we estimated the secreted salivary prion dose over a 10-mo period to be
as high as 8.4 log ID50 units for sheep and 7.0 log ID50 units for deer. These
estimates are similar to 7.9 log ID50 units of fecal CWD prions for deer.
Because saliva is mostly swallowed, salivary prions may reinfect tissues of the
gastrointestinal tract and contribute to fecal prion shedding. Salivary prions
shed into the environment provide an additional mechanism for horizontal prion
transmission.
ALSO, NOTE MINERAL LICKS A POSSIBLE SOURCE AND TRANSMISSION MODE FOR CWD
Elk and Deer Use of Mineral Licks: Implications for Disease Transmission
Kurt C. VerCauteren1*, Michael J. Lavelle1, Gregory E. Phillips1, Justin W.
Fischer1, and Randal S. Stahl1 1United States Department of Agriculture, Animal
and Plant Health Inspection Service, Wildlife Services, National Wildlife
Research Center, 4101 LaPorte Avenue, Fort Collins, CO 80521-2154, USA
*Cooresponding author e-mail: kurt.c.vercauteren@aphis.usda.gov
North American cervids require and actively seek out minerals to satisfy
physiological requirements. Minerals required by free-ranging cervids exist
within natural and artificial mineral licks that commonly serve as focal sites
for cervids. Ingestion of soils contaminated with the agent that causes chronic
wasting disease (CWD) may result in risk of contracting CWD. Our objective was
to evaluate the extent and nature of use of mineral licks by CWD-susceptible
cervid species. We used animal-activated cameras to monitor use of 18 mineral
licks between 1 June and 16 October 2006 in Rocky Mountain National Park,
north-central Colorado. We also assessed mineral concentrations at mineral licks
to evaluate correlations between visitation rates and site-specific
characteristics. We collected > 400,000 images of which 991 included elk, 293
included deer, and 6 included moose. We documented elk and deer participating in
a variety of potentially risky behaviors (e.g., ingesting soil, ingesting water,
defecating, urinating) while at mineral licks. 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.
PO-081: Chronic wasting disease in the cat— Similarities to feline
spongiform encephalopathy (FSE)
Thursday, May 31, 2012
CHRONIC WASTING DISEASE CWD PRION2012 Aerosol, Inhalation transmission,
Scrapie, cats, species barrier, burial, and more
UPDATED DATA ON 2ND CWD STRAIN Wednesday, September 08, 2010 CWD PRION
CONGRESS SEPTEMBER 8-11 2010
Tuesday, January 10, 2012
ESHRE position statement concerning prion detection in urinary gonadotropin
formulations
Prion Disease Detection, PMCA Kinetics, and IgG in Urine from Sheep
Naturally/Experimentally Infected with Scrapie and Deer with
Preclinical/Clinical Chronic Wasting Disease
▿Richard Rubenstein1,*, Binggong Chang1, Perry Gray2, Martin Piltch2, Marie
S. Bulgin3, Sharon Sorensen-Melson3 and Michael W. Miller4 + Author Affiliations
1Departments of Neurology and Physiology/Pharmacology, SUNY Downstate
Medical Center, 450 Clarkson Avenue, Brooklyn, New York 11203 2Los Alamos
National Laboratory, Los Alamos, New Mexico 87545 3University of Idaho, Caine
Veterinary Teaching and Research Center, 1020 E. Homedale Road, Caldwell, Idaho
83607 4Colorado Division of Wildlife, Wildlife Research Center, 317 West
Prospect Road, Fort Collins, Colorado 80526-2097
ABSTRACT
Prion diseases, also known as transmissible spongiform encephalopathies,
are fatal neurodegenerative disorders. Low levels of infectious agent and
limited, infrequent success of disease transmissibility and PrPSc detection have
been reported with urine from experimentally infected clinical cervids and
rodents. We report the detection of prion disease-associated seeding activity
(PASA) in urine from naturally and orally infected sheep with clinical scrapie
agent and orally infected preclinical and infected white-tailed deer with
clinical chronic wasting disease (CWD). This is the first report on PASA
detection of PrPSc from the urine of naturally or preclinical prion-diseased
ovine or cervids. Detection was achieved by using the surround optical fiber
immunoassay (SOFIA) to measure the products of limited serial protein misfolding
cyclic amplification (sPMCA). Conversion of PrPC to PrPSc was not influenced by
the presence of poly(A) during sPMCA or by the homogeneity of the PrP genotypes
between the PrPC source and urine donor animals. Analysis of the sPMCA-SOFIA
data resembled a linear, rather than an exponential, course. Compared to
uninfected animals, there was a 2- to 4-log increase of proteinase K-sensitive,
light chain immunoglobulin G (IgG) fragments in scrapie-infected sheep but not
in infected CWD-infected deer. The higher-than-normal range of IgG levels found
in the naturally and experimentally infected clinical scrapie-infected sheep
were independent of their genotypes. Although analysis of urine samples
throughout the course of infection would be necessary to determine the
usefulness of altered IgG levels as a disease biomarker, detection of PrPSc from
PASA in urine points to its potential value for antemortem diagnosis of prion
diseases.
FOOTNOTES
Received 13 May 2011. Accepted 14 June 2011. ↵*Corresponding author.
Mailing address: Downstate Medical Center, Departments of Neurology and
Physiology/Pharmacology, Box 1213, 450 Clarkson Avenue, Brooklyn, NY 11203.
Phone: (718) 270-2019. Fax: (718) 270-2459. E-mail:
richard.rubenstein@downstate.edu. ↵▿ Published ahead of print on 29 June 2011.
Copyright © 2011, American Society for Microbiology. All Rights Reserved.
Sunday, July 03, 2011
Prion Disease Detection, PMCA Kinetics, and IgG in Urine from
Naturally/Experimentally Infected Scrapie Sheep and Preclinical/Clinical CWD
Deer
Thursday, June 09, 2011
Detection of CWD prions in salivary, urinary, and intestinal tissues of
deer: potential mechanisms of prion shedding and transmission
CHRONIC WASTING DISEASE: A MODEL FOR PRION TRANSMISSION VIA SALIVA AND
URINE
Sunday, December 06, 2009
Detection of Sub-Clinical CWD Infection in Conventional Test-Negative Deer
Long after Oral Exposure to Urine and Feces from CWD+ Deer
Wednesday, March 18, 2009
Detection of CWD Prions in Urine and Saliva of Deer by Transgenic Mouse
Bioassay
*** Tuesday, September 02, 2008
Detection of infectious prions in urine (Soto et al Available online 13
August 2008.)
-------- Original Message --------
Subject: MAD DEER FEED BAN WARNING LETTER RECALL 6 TONS DISTRIBUTED USA
Date: Wed, 20 Oct 2004 14:53:56 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@UNI-KARLSRUHE.DE
##################### Bovine Spongiform Encephalopathy
#####################
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
#################
-------- Original Message --------
Subject: 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
From: "Terry S. Singeltary Sr."
To: fdadockets@oc.fda.gov
Greetings FDA,
i would kindly like to comment on;
Docket 03D-0186
FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal
Feed; Availability
Several factors on this apparent voluntary proposal disturbs me greatly,
please allow me to point them out;
1. MY first point is the failure of the partial ruminant-to-ruminant feed
ban of 8/4/97. this partial and voluntary feed ban of some ruminant materials
being fed back to cattle is terribly flawed. without the _total_ and _mandatory_
ban of all ruminant materials being fed back to ruminants including cattle,
sheep, goat, deer, elk and mink, chickens, fish (all farmed animals for
human/animal consumption), this half ass measure will fail terribly, as in the
past decades...
2. WHAT about sub-clinical TSE in deer and elk? with the recent findings of
deer fawns being infected with CWD, how many could possibly be sub-clinically
infected. until we have a rapid TSE test to assure us that all deer/elk are free
of disease (clinical and sub-clinical), we must ban not only documented CWD
infected deer/elk, but healthy ones as well. it this is not done, they system
will fail...
3. WE must ban not only CNS (SRMs specified risk materials), but ALL
tissues. recent new and old findings support infectivity in the rump or ass
muscle. wether it be low or high, accumulation will play a crucial role in TSEs.
4. THERE are and have been for some time many TSEs in the USA. TME in mink,
Scrapie in Sheep and Goats, and unidentified TSE in USA cattle. all this has
been proven, but the TSE in USA cattle has been totally ignored for decades. i
will document this data below in my references.
5. UNTIL we ban all ruminant by-products from being fed back to ALL
ruminants, until we rapid TSE test (not only deer/elk) but cattle in sufficient
numbers to find (1 million rapid TSE test in USA cattle annually for 5 years),
any partial measures such as the ones proposed while ignoring sub-clinical TSEs
and not rapid TSE testing cattle, not closing down feed mills that continue to
violate the FDA's BSE feed regulation (21 CFR 589.2000) and not making freely
available those violations, will only continue to spread these TSE mad cow
agents in the USA. I am curious what we will call a phenotype in a species that
is mixed with who knows how many strains of scrapie, who knows what strain or
how many strains of TSE in USA cattle, and the CWD in deer and elk (no telling
how many strains there), but all of this has been rendered for animal feeds in
the USA for decades. it will get interesting once someone starts looking in all
species, including humans here in the USA, but this has yet to happen...
6. IT is paramount that CJD be made reportable in every state (especially
''sporadic'' cjd), and that a CJD Questionnaire must be issued to every family
of a victim of TSE. only checking death certificates will not be sufficient.
this has been proven as well (see below HISTORY OF CJD -- CJD QUESTIONNAIRE)
7. WE must learn from our past mistakes, not continue to make the same
mistakes...
snip...
Oral transmission and early lymphoid tropism of chronic wasting disease
PrPres in mule deer fawns (Odocoileus hemionus )
Christina J. Sigurdson1, Elizabeth S. Williams2, Michael W. Miller3, Terry
R. Spraker1,4, Katherine I. O'Rourke5 and Edward A. Hoover1
Department of Pathology, College of Veterinary Medicine and Biomedical
Sciences, Colorado State University, Fort Collins, CO 80523- 1671, USA1
Department of Veterinary Sciences, University of Wyoming, 1174 Snowy Range Road,
University of Wyoming, Laramie, WY 82070, USA 2 Colorado Division of Wildlife,
Wildlife Research Center, 317 West Prospect Road, Fort Collins, CO 80526-2097,
USA3 Colorado State University Veterinary Diagnostic Laboratory, 300 West Drake
Road, Fort Collins, CO 80523-1671, USA4 Animal Disease Research Unit,
Agricultural Research Service, US Department of Agriculture, 337 Bustad Hall,
Washington State University, Pullman, WA 99164-7030, USA5
Author for correspondence: Edward Hoover.Fax +1 970 491 0523. e-mail
ehoover@lamar.colostate.edu
Mule deer fawns (Odocoileus hemionus) were inoculated orally with a brain
homogenate prepared from mule deer with naturally occurring chronic wasting
disease (CWD), a prion-induced transmissible spongiform encephalopathy. Fawns
were necropsied and examined for PrP res, the abnormal prion protein isoform, at
10, 42, 53, 77, 78 and 80 days post-inoculation (p.i.) using an
immunohistochemistry assay modified to enhance sensitivity. PrPres was detected
in alimentary-tract-associated lymphoid tissues (one or more of the following:
retropharyngeal lymph node, tonsil, Peyer's patch and ileocaecal lymph node) as
early as 42 days p.i. and in all fawns examined thereafter (53 to 80 days p.i.).
No PrPres staining was detected in lymphoid tissue of three control fawns
receiving a control brain inoculum, nor was PrPres detectable in neural tissue
of any fawn. PrPres-specific staining was markedly enhanced by sequential tissue
treatment with formic acid, proteinase K and hydrated autoclaving prior to
immunohistochemical staining with monoclonal antibody F89/160.1.5. 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 results indicate that mule deer fawns develop detectable PrP res
after oral exposure to an inoculum containing CWD prions. In the earliest
post-exposure period, CWD PrPres was traced to the lymphoid tissues draining the
oral and intestinal mucosa (i.e. the retropharyngeal lymph nodes, tonsil, ileal
Peyer's patches and ileocaecal lymph nodes), which probably received the highest
initial exposure to the inoculum. Hadlow et al. (1982) demonstrated scrapie
agent in the tonsil, retropharyngeal and mesenteric lymph nodes, ileum and
spleen in a 10-month-old naturally infected lamb by mouse bioassay. Eight of
nine sheep had infectivity in the retropharyngeal lymph node. He concluded that
the tissue distribution suggested primary infection via the gastrointestinal
tract. The tissue distribution of PrPres in the early stages of infection in the
fawns is strikingly similar to that seen in naturally infected sheep with
scrapie. 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...
===================================
now, just what is in that deer feed? _ANIMAL PROTEIN_
Subject: MAD DEER/ELK DISEASE AND POTENTIAL SOURCES
Date: Sat, 25 May 2002 18:41:46 –0700
From: "Terry S. Singeltary Sr."
Reply-To: BSE-L To: BSE-L
8420-20.5% Antler Developer For Deer and Game in the wild Guaranteed
Analysis Ingredients / Products Feeding Directions
snip...
_animal protein_
snip...
PLEASE SEE FULL TEXT SUBMISSION ;
Subject: 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
From: "Terry S. Singeltary Sr."
CWD TO HUMAN TRANSMISSION, never say never !!!
Envt.06:
Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates
Emmanuel Comoy,1,† Valérie Durand,1 Evelyne Correia,1 Aru Balachandran,2
Jürgen Richt,3 Vincent Beringue,4 Juan-Maria Torres,5 Paul Brown,1 Bob Hills6
and Jean-Philippe Deslys1
1Atomic Energy Commission; Fontenay-aux-Roses, France; 2Canadian Food
Inspection Agency; Ottawa, ON Canada; 3Kansas State University; Manhattan, KS
USA; 4INRA; Jouy-en-Josas, France; 5INIA; Madrid, Spain; 6Health Canada; Ottawa,
ON Canada
†Presenting author; Email: emmanuel.comoy@cea.fr
The constant increase of chronic wasting disease (CWD) incidence in North
America raises a question about their zoonotic potential. A recent publication
showed their transmissibility to new-world monkeys, but no transmission to
old-world monkeys, which are phylogenetically closer to humans, has so far been
reported. Moreover, several studies have failed to transmit CWD to transgenic
mice overexpressing human PrP. Bovine spongiform encephalopathy (BSE) is the
only animal prion disease for which a zoonotic potential has been proven. We
described the transmission of the atypical BSE-L strain of BSE to cynomolgus
monkeys, suggesting a weak cattle-to-primate species barrier. We observed the
same phenomenon with a cattleadapted strain of TME (Transmissible Mink
Encephalopathy). Since cattle experimentally exposed to CWD strains have also
developed spongiform encephalopathies, we inoculated brain tissue from
CWD-infected cattle to three cynomolgus macaques as well as to transgenic mice
overexpressing bovine or human PrP. Since CWD prion strains are highly
lymphotropic, suggesting an adaptation of these agents after peripheral
exposure, a parallel set of four monkeys was inoculated with CWD-infected cervid
brains using the oral route. Nearly four years post-exposure, monkeys exposed to
CWD-related prion strains remain asymptomatic. In contrast, bovinized and
humanized transgenic mice showed signs of infection, suggesting that CWD-related
prion strains may be capable of crossing the cattle-to-primate species barrier.
Comparisons with transmission results and incubation periods obtained after
exposure to other cattle prion strains (c-BSE, BSE-L, BSE-H and cattle-adapted
TME) will also be presented, in order to evaluate the respective risks of each
strain.
Envt.07:
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free
Ranging White-Tailed Deer Infected with Chronic Wasting Disease
Martin L. Daus,1,† Johanna Breyer,2 Katjs Wagenfuehr,1 Wiebke Wemheuer,2
Achim Thomzig,1 Walter Schulz-Schaeffer2 and Michael Beekes1 1Robert Koch
Institut; P24 TSE; Berlin, Germany; 2Department of Neuropathology, Prion and
Dementia Research Unit, University Medical Center Göttingen; Göttingen, Germany
†Presenting author; Email: dausm@rki.de
Chronic wasting disease (CWD) is a contagious, rapidly spreading
transmissible spongiform encephalopathy (TSE) occurring in cervids in North
America. Despite efficient horizontal transmission of CWD among cervids natural
transmission of the disease to other species has not yet been observed. Here, we
report a direct biochemical demonstration of pathological prion protein PrPTSE
and of PrPTSE-associated seeding activity in skeletal muscles of CWD-infected
cervids. The presence of PrPTSE was detected by Western- and postfixed frozen
tissue blotting, while the seeding activity of PrPTSE was revealed by protein
misfolding cyclic amplification (PMCA). The concentration of PrPTSE in skeletal
muscles of CWD-infected WTD was estimated to be approximately 2000- to
10000-fold lower than in brain tissue. Tissue-blot-analyses revealed that PrPTSE
was located in muscle- associated nerve fascicles but not, in detectable
amounts, in myocytes. 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.
PLUS, THE CDC DID NOT PUT THIS WARNING OUT FOR THE WELL BEING OF THE DEER
AND ELK ;
Thursday, May 26, 2011
Travel History, Hunting, and Venison Consumption Related to Prion Disease
Exposure, 2006-2007 FoodNet Population Survey
Journal of the American Dietetic Association Volume 111, Issue 6 , Pages
858-863, June 2011.
NOR IS THE FDA recalling this CWD positive elk meat for the well being of
the dead elk ;
Wednesday, March 18, 2009
Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat
derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS
AND FIELD CORRECTIONS: FOODS CLASS II
Sunday, January 22, 2012
Chronic Wasting Disease CWD cervids interspecies transmission
now, let’s see what the authors said about this casual link, personal
communications years ago. see where it is stated NO STRONG evidence. so, does
this mean there IS casual evidence ????
“Our conclusion stating that we found no strong evidence of CWD
transmission to humans”
From: TSS (216-119-163-189.ipset45.wt.net)
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias"
To:
Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam,
In the Archives of Neurology you quoted (the abstract of which was attached
to your email), we did not say CWD in humans will present like variant CJD.
That assumption would be wrong. I encourage you to read the whole article
and call me if you have questions or need more clarification (phone:
404-639-3091). Also, we do not claim that "no-one has ever been infected with
prion disease from eating venison." Our conclusion stating that we found no
strong evidence of CWD transmission to humans in the article you quoted or in
any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message-----
From:
Sent: Sunday, September 29, 2002 10:15 AM
To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease
2008 1: Vet Res. 2008 Apr 3;39(4):41
A prion disease of cervids: Chronic wasting disease
Sigurdson CJ.
snip...
*** twenty-seven CJD patients who regularly consumed venison were reported
to the Surveillance Center***,
snip...
full text ;
Sunday, January 22, 2012
Chronic Wasting Disease CWD cervids interspecies transmission
AS THE CROW FLIES, SO DOES CWD
Sunday, November 01, 2009
American crows (Corvus brachyrhynchos) and potential spreading of CWD
through feces of digested infectious carcases
Wednesday, October 17, 2012
Prion Remains Infectious after Passage through Digestive System of American
Crows (Corvus brachyrhynchos)
LANCET INFECTIOUS DISEASE JOURNAL
Volume 3, Number 8 01 August 2003
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever
since. What I have found is that we have not been told the truth. CWD in deer
and elk is a small portion of a much bigger problem.
49-year-old Singeltary is one of a number of people who have remained
largely unsatisfied after being told that a close relative died from a rapidly
progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease
(CJD). So he decided to gather hundreds of documents on transmissible spongiform
encephalopathies (TSE) and realised that if Britons could get variant CJD from
bovine spongiform encephalopathy (BSE), Americans might get a similar disorder
from chronic wasting disease (CWD)the relative of mad cow disease seen among
deer and elk in the USA. Although his feverish search did not lead him to the
smoking gun linking CWD to a similar disease in North American people, it did
uncover a largely disappointing situation.
Singeltary was greatly demoralised at the few attempts to monitor the
occurrence of CJD and CWD in the USA. Only a few states have made CJD
reportable. Human and animal TSEs should be reportable nationwide and
internationally, he complained in a letter to the Journal of the American
Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue
to expect us to still believe that the 85% plus of all CJD cases which are
sporadic are all spontaneous, without route or source.
Until recently, CWD was thought to be confined to the wild in a small
region in Colorado. But since early 2002, it has been reported in other areas,
including Wisconsin, South Dakota, and the Canadian province of Saskatchewan.
Indeed, the occurrence of CWD in states that were not endemic previously
increased concern about a widespread outbreak and possible transmission to
people and cattle.
To date, experimental studies have proven that the CWD agent can be
transmitted to cattle by intracerebral inoculation and that it can cross the
mucous membranes of the digestive tract to initiate infection in lymphoid tissue
before invasion of the central nervous system. Yet the plausibility of CWD
spreading to people has remained elusive.
Getting data on TSEs in the USA from the government is like pulling teeth,
Singeltary argues. You get it when they want you to have it, and only what they
want you to have.
SNIP...FULL TEXT ;
Friday, October 26, 2012
*** CHRONIC WASTING DISEASE CWD PENNSYLVANIA GAME FARMS, URINE ATTRACTANT
PRODUCTS, BAITING, AND MINERAL LICKS
Friday, November 09, 2012
Chronic Wasting Disease CWD in cervidae and transmission to other species
Sunday, November 11, 2012
Susceptibilities of Nonhuman Primates to Chronic Wasting Disease November
2012
see towards the bottom, Soto et al study, and the young people that
consumed cervids, that died from CJD? all coincidence per cdc et al. I remember
when the UK said that about CJD with young folks and BSE aka mad cow disease. it
took ten years for the officials and the industry to finally admit to that. even
then, some of the industry still does not believe it $. I remember john gummer
force feeding his daughter a hamburger, trying to show UK beef was safe (when it
was not), and then about 10 years later, his daughter close friend died from CJD
;
let’s pray we do not go down that same road here in the USA...see full text
;
Sunday, November 11, 2012
Susceptibilities of Nonhuman Primates to Chronic Wasting Disease November
2012
Generation of a new form of human PrPSc in vitro by inter-species
transmission from cervids prions
Marcelo A. Barria1, Glenn C. Telling2, Pierluigi Gambetti3, James A.
Mastrianni4 and Claudio Soto1,* 1Mitchell Center for Alzheimer’s disease and
related Brain disorders, Dept of Neurology, University of Texas Houston Medical
School, Houston, TX 77030, USA 2Dept of Microbiology, Immunology & Molecular
Genetics, and Neurology, Sanders Brown Center on Aging, University of Kentucky
Medical Center, Lexington, KY, USA 3Institute of Pathology, Case Western Reserve
University, Cleveland, OH, USA 4Dept of Neurology, University of Chicago,
Chicago, IL, USA. Running Title: Conversion of human PrPC by cervid PrPSc
Keywords: Prion / transmissible spongiform encephalopathy / infectivity /
misfolded prion protein / prion strains * To whom correspondence should be
addressed. University of Texas Houston Medical School, 6431 Fannin St, Houston,
TX 77030. Tel 713-5007086; Fax 713-5000667; E-mail Claudio.Soto@uth.tmc.edu The
latest version is at http://www.jbc.org/cgi/doi/10.1074/jbc.M110.198465
JBC Papers in Press.
Published on January 4, 2011 as Manuscript M110.198465 Copyright 2011 by
The American Society for Biochemistry and Molecular Biology, Inc. 5, Downloaded
from www.jbc.org by guest, on November 11, 2012 2
Prion diseases are infectious neurodegenerative disorders affecting humans
and animals that result from the conversion of normal prion protein (PrPC) into
the misfolded prion protein (PrPSc). Chronic wasting disease (CWD) is a prion
disorder of increasing prevalence within the United States that affects a large
population of wild and captive deer and elk. Determining the risk of
transmission of CWD to humans is of utmost importance, considering that people
can be infected by animal prions, resulting in new fatal diseases. To study the
possibility that human PrPC can be converted into the misfolded form by CWD
PrPSc we performed experiments using the Protein Misfolding Cyclic Amplification
(PMCA) technique, which mimic in vitro the process of prion replication. Our
results show that cervid PrPSc can induce the conversion of human PrPC, but only
after the CWD prion strain has been stabilized by successive passages in vitro
or in vivo. Interestingly, the newly generated human PrPSc exhibits a distinct
biochemical pattern that differs from any of the currently known forms of human
PrPSc. Our results also have profound implications for understanding the
mechanisms of prion species barrier and indicate that the transmission barrier
is a dynamic process that depend on the strain and moreover the degree of
adaptation of the strain. If our findings are corroborated by infectivity
assays, they will imply that CWD prions have the potential to infect humans, and
that this ability depends on CWD strain adaptation.
Various studies aimed to analyze the transmission of CWD to transgenic mice
expressing human PrP have consistently given negative results (9-11), indicating
a strong species barrier. This conclusion is consistent with our many failed
experiments to attempt converting human PrPC with natural CWD, even after
pushing the PMCA conditions (see figure 1). We found successful conversion only
after adaptation of the CWD prion strain by successive passages in vitro or in
cervid transgenic mice. We are not aware that in any of the transgenic mice
studies the inoculum used was a previously stabilized CWD strain. Although, it
has been shown that strain stabilization in vitro by PMCA (17;26) and in vivo
using experimental rodents (36) has similarities with the strain adaptation
process occurring in natural hosts, we cannot rule out that the type of CWD
strain adaptation that is required to produce strains transmissible to humans
may take much longer time in cervids or not occur at all. An important
experiment will be to study transmissibility to humanized transgenic mice of CWD
passed experimentally in deer several times. Besides the importance of our
results for public health in relation to the putative transmissibility of CWD to
humans, our data also illustrate a very important and novel scientific concept
related to the mechanism of prion transmission across species barriers. Today
the view is that species barrier is mostly controlled by the degree of
similarity on the sequence of the prion protein between the host and the
infectious material (4). In our study we show that the strain and moreover the
stabilization of the strain plays a major role in the inter-species
transmission. In our system there is no change on the protein sequence, but yet
strain adaptation results in a complete change on prion transmissibility with
potentially dramatic consequences. Therefore, our findings lead to a new view of
the species barrier that should not be seen as a static process, but rather a
dynamic biological phenomenon that can change over time when prion strains
mature and evolve. It remains to be investigated if other species barriers also
change upon progressive strain adaptation of other prion forms (e.g. the
sheep/human barrier).
Our results have far-reaching implications for human health, since they
indicate that cervid PrPSc can trigger the conversion of human PrPC into PrPSc,
suggesting that CWD might be infectious to humans. Interestingly our findings
suggest that unstable strains from CWD affected animals might not be a problem
for humans, but upon strain stabilization by successive passages in the wild,
this disease might become progressively more transmissible to man.
Generation of a New Form of Human PrPScin Vitro by Interspecies
Transmission from Cervid Prions*
Marcelo A. Barria‡, Glenn C. Telling§, Pierluigi Gambetti¶, James A.
Mastrianni‖ and Claudio Soto‡,1 + Author Affiliations
From the ‡Mitchell Center for Alzheimer's Disease and Related Brain
Disorders, Department of Neurology, University of Texas Medical School at
Houston, Houston, Texas 77030, the §Departments of Microbiology, Immunology, and
Molecular Genetics and Neurology, Sanders Brown Center on Aging, University of
Kentucky Medical Center, Lexington, Kentucky 40506, the ¶Institute of Pathology,
Case Western Reserve University, Cleveland, Ohio 44106, and the ‖Department of
Neurology, The University of Chicago, Chicago, Illinois 60637 1 To whom
correspondence should be addressed: University of Texas Medical School at
Houston, 6431 Fannin St., Houston, TX 77030. Tel.: 713-500-7086; Fax:
713-500-0667; E-mail: claudio.soto@uth.tmc.edu.
Abstract
Prion diseases are infectious neurodegenerative disorders that affect
humans and animals and that result from the conversion of normal prion protein
(PrPC) into the misfolded prion protein (PrPSc). Chronic wasting disease (CWD)
is a prion disorder of increasing prevalence within the United States that
affects a large population of wild and captive deer and elk. Determining the
risk of transmission of CWD to humans is of utmost importance, considering that
people can be infected by animal prions, resulting in new fatal diseases. To
study the possibility that human PrPC can be converted into the misfolded form
by CWD PrPSc, we performed experiments using the protein misfolding cyclic
amplification technique, which mimics in vitro the process of prion replication.
Our results show that cervid PrPSc can induce the conversion of human PrPC but
only after the CWD prion strain has been stabilized by successive passages in
vitro or in vivo. Interestingly, the newly generated human PrPSc exhibits a
distinct biochemical pattern that differs from that of any of the currently
known forms of human PrPSc. Our results also have profound implications for
understanding the mechanisms of the prion species barrier and indicate that the
transmission barrier is a dynamic process that depends on the strain and
moreover the degree of adaptation of the strain. If our findings are
corroborated by infectivity assays, they will imply that CWD prions have the
potential to infect humans and that this ability progressively increases with
CWD spreading.
UPDATED DATA ON 2ND CWD STRAIN
Wednesday, September 08, 2010
CWD PRION CONGRESS SEPTEMBER 8-11 2010
OR-10: Variably protease-sensitive prionopathy is transmissible in bank
voles
Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1
Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan
Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome,
Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna,
Italy; 3Case Western Reserve University; Cleveland, OH USA
Background. Variably protease-sensitive prionopathy (VPSPr) is a recently
described “sporadic”neurodegenerative disease involving prion protein
aggregation, which has clinical similarities with non-Alzheimer dementias, such
as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in
Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the
prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is
the electrophoretic pattern of PrPSc after digestion with proteinase K (PK).
After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern
similar to that described in GSS cases. The clinical and pathological features
of VPSPr raised the question of the correct classification of VPSPr among prion
diseases or other forms of neurodegenerative disorders. Here we report
preliminary data on the transmissibility and pathological features of VPSPr
cases in bank voles.
Materials and Methods. Seven VPSPr cases were inoculated in two genetic
lines of bank voles, carrying either methionine or isoleucine at codon 109 of
the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases
selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical
diagnosis in voles was confirmed by brain pathological assessment and western
blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.
Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission
in BvM109. Overall, 3 voles were positive with survival time between 290 and 588
d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form
of the typical PrP27–30, which was indistinguishable to that previously observed
in BvM109 inoculated with sCJDMM1 cases.
In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until
now. Overall, 5 voles were positive with survival time between 281 and 596
d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like
PrPSc electrophoretic pattern, characterized by low molecular weight PrPres.
These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative
with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus
and the N-terminus. Second passages are in progress from these first successful
transmissions.
Conclusions. Preliminary results from transmission studies in bank voles
strongly support the notion that VPSPr is a transmissible prion disease.
Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of
voles, with sCJD-like features in BvM109 and GSS-like properties in
BvI109.
The discovery of previously unrecognized prion diseases in both humans and
animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion
diseases might be wider than expected and raises crucial questions about the
epidemiology and strain properties of these new forms. We are investigating this
latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.
*** The discovery of previously unrecognized prion diseases in both humans
and animals (i.e., Nor98 in small ruminants) demonstrates that the range of
prion diseases might be wider than expected and raises crucial questions about
the epidemiology and strain properties of these new forms. We are investigating
this latter issue by molecular and biological comparison of VPSPr, GSS and
Nor98.
AS OF AUGUST 2012 ;
CJD UPDATE USA
1 Listed based on the year of death or, if not available, on year of
referral; 2 Cases with suspected prion disease for which brain tissue and/or
blood (in familial cases) were submitted; 3 Disease acquired in the United
Kingdom; 4 Disease was acquired in the United Kingdom in one case and in Saudi
Arabia in the other case; *** 5 Includes 8 cases in which the diagnosis is
pending, and 18 inconclusive cases; *** 6 Includes 10 (9 from 2012) cases with
type determination pending in which the diagnosis of vCJD has been excluded. ***
The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI) and 42
cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2224 cases of
sporadic Creutzfeldt-Jakob disease (sCJD).
Wednesday, March 28, 2012
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion
poker goes up again $
Saturday, October 6, 2012
*** TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE
SPONGIFORM ENCEPHALOPATHIES 2011 Annual Report
Friday, February 03, 2012
Wisconsin Farm-Raised Deer Farms and CWD there from 2012 report Singeltary
et al
Thursday, February 09, 2012
Colorado Farm-Raised Deer Farms and CWD there from 2012 report Singeltary
et al
Tuesday, February 14, 2012
Oppose Indiana House Bill 1265 game farming cervids
Monday, February 13, 2012
Stop White-tailed Deer Farming from Destroying Tennessee’s Priceless Wild
Deer Herd oppose HB3164
Wednesday, February 15, 2012
West Virginia Deer Farming Bill backed by deer farmers advances, why ? BE
WARNED CWD
Wednesday, February 29, 2012
Sen. Tommy Gollott Mississippi proposes another bill to allow CWD in
Mississippi via Game Farms
Wednesday, March 21, 2012
MICHIGAN SENATE BILL 27 TURNS OVER GAME FARMS and CWD RISK FACTORS THERE
FROM, TO DEPARTMENT OF AGRICULTURE $
Friday, March 16, 2012
OHIO TURNS OVER CERVID GAME FARMS (and CWD risk) TO DEPARTMENT OF
AGRICULTURE, GOD HELP THEM
As Passed by the Senate
129th General Assembly Regular Session 2011-2012 Am. H. B. No. 389
Ohio ranks #3 in Deer and Elk Farms 2010
Deer farms in 82 of 88 counties in Ohio
Ohio’s Fatal Attractions
An overview of captive wildlife issues in Ohio
April 4, 2011
Updated March 20, 2012
Monday, June 11, 2012
OHIO Captive deer escapees and non-reporting
Saturday, February 04, 2012
Wisconsin 16 age limit on testing dead deer Game Farm CWD Testing Protocol
Needs To Be Revised
Friday, June 01, 2012
TEXAS DEER CZAR TO WISCONSIN ASK TO EXPLAIN COMMENTS
Wednesday, January 07, 2009
CWD to tighten taxidermy rules Hunters need to understand regulations
TSS
layperson
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net
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