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From: TSS ()
Subject: CWD UPDATE 88 AUGUST 31, 2007
Date: September 1, 2007 at 11:25 am PST

CWD Update 88


August 31, 2007


State and Provincial Updates

Illinois:

Paul Shelton, Illinois Department of Natural Resources provides the following: During July,
IDNR identified a CWD-positive deer in LaSalle County after testing an animal showing classic
signs of the illness. This was the first instance of the disease in this county. The deer was a 3
year old doe collected by a Conservation Police Officer after someone reported a sick, emaciated
deer. The location was south of I-80, about 2 miles west of Grundy County, near the town of
Seneca. This represents about a 25 mile distance from the previous southernmost positive in
DeKalb County. Staff from the Division of Wildlife Resources are assessing the implications of
the finding.

The total number of CWD-infected deer found in Illinois now numbers 189. Prior to this, the
disease had been confined to Winnebago, Boone, McHenry, Ogle, and DeKalb counties. More
than 28,000 deer have been tested in Illinois during the past 5 years. Illinois DNR CWD
information is available at: http://dnr.state.il.us/cwd.
Editor’s note: This finding in LaSalle County is a significant departure from the previously
known distribution in Illinois. The new location is the first deer detected in the Illinois River
basin, which winds southwest through Illinois towards St. Louis.

New Mexico:

Press Release from New Mexico Game and Fish (August 28, 2007):

LAS CRUCES: New Mexico recorded its 19th case of chronic wasting disease in deer in a sick
animal found in the Bishop's Cap area of the Organ Mountains.
Officer Richard McDonald investigated a report of an emaciated deer July 12. The animal was
unaware of human presence, chronically thirsty, urinating often, and staying in and near a water
source. Officer McDonald followed the state's protocol for disease surveillance by killing the
animal and sending it to the Veterinary Diagnostic Laboratory in Albuquerque for testing.
Based on the symptoms and the area from which the deer came, the laboratory was instructed
that chronic wasting disease (CWD) was highly probable. Laboratory diagnostic testing
confirmed presence of CWD in this deer. This is the 19th deer with confirmed CWD found since
it was first detected in New Mexico in 2002. Two elk have also been found with CWD.
This deer was in Game Management Unit 19, where special CWD restrictions already exist for
hunters.
Anyone who finds a deer or elk that appears unaware of human presence and displays symptoms
including droopy ears, emaciation, chronic thirst, frequent urination, and reluctance to leave
water, should report their observations to the Department of Game and Fish, Wildlife
Management Division, (505) 476-8127.
New Mexico Game & Fish CWD information is at:
http://www.wildlife.state.nm.us/conservation/disease/cwd/index.htm.
Press Release is at:
http://www.wildlife.state.nm.us/publications/press_releases/documents/2007/082807releases.htm


#CWD.

Recent Publications

Efficient In Vitro Amplification of Chronic Wasting Disease PrPRES
Timothy D. Kurt, Matthew R. Perrott, Carol J. Wilusz, Jeffrey Wilusz, Surachai Supattapone,
Glenn C. Telling, Mark D. Zabel, and Edward A. Hoover
Journal of Virology, September 2007, p. 9605-9608, Vol. 81, No. 17

Abstract: Chronic wasting disease (CWD) of cervids is associated with conversion of the normal
cervid prion protein, PrPC, to a protease-resistant conformer, PrPCWD. Here we report the use of
both nondenaturing amplification and protein-misfolding cyclic amplification (PMCA) to
amplify PrPCWD in vitro. Normal brains from deer, transgenic mice expressing cervid PrPC
[Tg(cerPrP)1536 mice], and ferrets supported amplification. PMCA using normal
Tg(cerPrP)1536 brains as the PrPC substrate produced >6.5 x 109-fold amplification after six
rounds. Highly efficient in vitro amplification of PrPCWD is a significant step toward detection of
PrPCWD in the body fluids or excreta of CWD-susceptible species.
http://jvi.asm.org/cgi/content/abstract/81/17/9605?etoc.

Ultrasensitive detection of scrapie prion protein using seeded conversion of recombinant
prion protein

Ryuichiro Atarashi, Roger A Moore, Valerie L Sim, Andrew G Hughson, David W Dorward,
Henry A Onwubiko, Suzette A Priola & Byron Caughey
Nature Methods - 4, 645 - 650 (2007)

Abstract: The scrapie prion protein isoform, PrPSc, is a prion-associated marker that seeds the
conformational conversion and polymerization of normal protease-sensitive prion protein (PrPsen).
This seeding activity allows ultrasensitive detection of PrPSc using cyclical sonicated
amplification (PMCA) reactions and brain homogenate as a source of PrP-sen. Here we describe
a much faster seeded polymerization method (rPrP-PMCA) which detects greater than or equal
to50 ag of hamster PrPSc (approximately0.003 lethal dose) within 2–3 d. This technique uses
recombinant hamster PrP-sen, which, unlike brain-derived PrP-sen, can be easily concentrated,
mutated and synthetically tagged. We generated protease-resistant recombinant PrP fibrils that
differed from spontaneously initiated fibrils in their proteolytic susceptibility and by their
infrared spectra. This assay could discriminate between scrapie-infected and uninfected hamsters
using 2-mul aliquots of cerebral spinal fluid. This method should facilitate the development of
rapid, ultrasensitive prion assays and diagnostic tests, in addition to aiding fundamental studies
of structure and mechanism of PrPSc formation.
http://www.nature.com/nmeth/journal/v4/n8/abs/nmeth1066.html.
The following two articles are from the June 2007 edition (Volume 1772, Issue 6) of the journal
Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease. This special edition of the
journal, edited by Glenn Telling, is devoted to prion-related disorders and is available at:
http://www.sciencedirect.com/science/journal/09254439.

Chronic Wasting Disease (review)

Christina J. Sigurdson and Adriano Aguzzi

Biochimica et Biophysica Acta 1772 (2007) 610–618

Abstract: Until recently, chronic wasting disease of cervids, the only prion disease affecting
wildlife, was believed to be geographically concentrated to Colorado and Wyoming within the
United States. However, increased surveillance has unveiled several additional pockets of CWDinfected
deer and elk in 12 additional states and 2 Canadian provinces. Deer and elk with CWD
have extensive aggregates of PrPSc not only in the central nervous system, but also in peripheral
lymphoid tissues, skeletal muscle, and other organs, perhaps influencing prion shedding. Indeed,
CWD is transmitted efficiently among animals by horizontal routes, although the mechanism of
spread is unknown. Genetic polymorphisms in the Prnp gene may affect CWD susceptibility,
particularly at codon 225 (S/F) in deer and codon 132 (M/L) in elk. Since CWD infects freeranging
animals and is efficiently spread, disease management will be a challenge.

Motor behavioral and neuropathological deficits in mice deficient for normal prion protein
expression

Karah E. Nazora, Tanya Sewarda and Glenn C. Telling
Biochimica et Biophysica Acta 1772 (2007) 645–653

Abstract: It has been difficult to reconcile the absence of pathology and apparently normal
behavior of mice lacking prion protein (PrP), referred to as Prnp0/0 mice, with a mechanism of
prion pathogenesis involving progressive loss of PrPC-mediated neuroprotection. However, here
we report that Prnp0/0 mice exhibit significant age-related defects in motor coordination and
balance compared with mice expressing wild type Prnp on a syngeneic background, and that the
brains of behaviorally-impaired Prnp0/0 mice display the cardinal neuropathological hallmarks of
spongiform pathology and reactive astrocytic gliosis that normally accompany prion disease.
Consistent with the appearance of cerebellar ataxia as an early symptom in patients with
Gerstmann–Sträussler–Scheinker syndrome (GSS), an inherited form of human prion disease,
motor coordination and balance defects manifested in a transgenic (Tg) mouse model of GSS
considerably earlier than the onset of end-stage neurodegenerative disease. Our results are
consistent with a mechanism in which loss of normal PrPC function is an important pathological
component of prion diseases.


http://wildlifedisease.nbii.gov/documents/CWD%20Updates/Report%2088.pdf

J Biol Chem. 2007 Aug 20; : 17709374

Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a
new prion strain.

[My paper] Atsushi Kobayashi , Masahiro Asano , Shirou Mohri , Tetsuyuki
Kitamoto

The genotype (methionine or valine) at polymorphic codon 129 of the human
prion protein (PrP) gene and the type (type 1 or type 2) of abnormal isoform
of PrP (PrP(Sc)) are major determinants of the clinicopathological
phenotypes of sporadic Creutzfeldt-Jakob disease (sCJD). Here we found that
transmission of sCJD prions from a patient with valine homozygosity (129V/V)
and type 2 PrP(Sc) (sCJD-VV2 prions) to mice expressing human PrP with
methionine homozygosity (129M/M) generated unusual PrP(Sc) intermediate in
size between type 1 and type 2. The intermediate type PrP(Sc) was seen in
all examined dura mater graft-associated CJD cases with 129M/M and
plaque-type PrP deposits (p-dCJD). p-dCJD prions and sCJD-VV2 prions
exhibited similar transmissibility and neuropathology, and the identical
type of PrP(Sc) when inoculated into PrP-humanized mice with 129M/M or
129V/V. These findings suggest that p-dCJD could be caused by cross-sequence
transmission of sCJD-VV2 prions.


snip...


In this study, the strain-dependent traits of sCJDMM1
prions were inherited through cross-sequence
transmission without any modification. The
humanized mice with 129V/V produced type 1 PrPres
after inoculation with sCJD-MM1 prions. Because
sCJD-VV1 cases are extremely rare (at most 1-2%
of the total number of sCJD cases) and characterized
by early onset (mean age at onset: 39.3 years) (5),

####################################

our results raise the possibility that CJD cases
classified as VV1 may include cases caused by
iatrogenic transmission of sCJD-MM1 prions or
food-borne infection by type 1 prions from animals,
e.g., chronic wasting disease prions in cervid. In fact,
two CJD-VV1 patients who hunted deer or
consumed venison have been reported (40, 41). The
results of the present study emphasize the need for
traceback studies and careful re-examination of the
biochemical properties of sCJD-VV1 prions.

###################################

In conclusion, cross-sequence transmission of
sCJD-VV2 prions generates a new prion strain with
altered conformational properties and disease
phenotypes as p-dCJD prions. Furthermore, the
newly generated prions have unique transmissibility
including the traceback phenomenon. In the future, if
atypical prion strains emerge through cross-sequence
transmission, especially from animals, traceback
studies will enable us to identify the origin of the
prions.

REFERENCES...snip...end

FULL TEXT ;


http://www.jbc.org/cgi/content/abstract/M704597200v1?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=Cross-sequence+transmission+of+sporadic+Creutzfeldt-Jakob+disease+creates+a+new+&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

Date: Wed, 29 Aug 2007 21:13:08 -0500
From: "Terry S. Singeltary Sr."
Subject: CWD NEW MEXICO RECORDS IT'S 19 CASE (near Texas border again)


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=26079


Subject: Monitoring the Potential Transmission of Chronic Wasting Disease to
Humans Using a Hunter Registry Database in Wyoming
Date: August 30, 2007 at 6:46 pm PST

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=27654


Re: Colorado Surveillance Program for Chronic Wasting Disease
Transmission to Humans (TWO SUSPECT CASES)


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0704&L=sanet-mg&T=0&P=1165


One reason for this was the _inaccuracy_ in coding of cases correctly
certified as CJD Coding is carried out by staff who are not medically
qualified and it is not surprising that coding errors occur in the
processing of large numbers of certificates. In 1982, 12,000
certificates per week were processed at the office of population
censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983).
The occurrence of both inter- and intra-observer coding errors has been
described (Curb et al., 1983) and the _inaccuracies_ of BOTH
certification and coding discovered in this study _support_ the
introduction of a more accurate system of death certificates and
a more detailed and specific coding system...

snip...

http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf


''Answering critics like Terry Singeltary, who feels that the US
undercounts CJD, Schonberger _conceded_ that the current surveillance
system has errors but stated that most of the errors will be confined to
the older population''...

The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly
Prion Diseases by Philip Yam

Philip Yam The Pathological Protein Mad Cow, Chronic Wasting, and Other
Deadly Prion Diseases 2003. Hardcover, 285 pp. Euro 29.95 (net price);
ã21.00; $27.50; sFr 51.50 ISBN 0-387-95508-9 Contact and review
copies: Joan Robinson Springer-Verlag Press and Public Relations Tel.:
+49- (0) 6221-487-8130, Fax: +49- (0) 6221-487-8141, E-mail:
[log in to unmask] http://www.springer.de/press/newbooks/protein.html
The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly
Prion Diseases Philip Yam List Price: $27.50 Our Price: $19.25 You Save:
$8.25 (30%) Availability: Usually ships within 24 hours.

http://www.target.com/gp/detail.html/sr=1-1/qid=1054420048/ref=sr_1_1/602-9780634-9614260?asin=0387955089


SAD THAT OLDER FOLKS SEEM TO BE EXPENDABLE IN RELATIONS TO TSEs.

IF YOUR NOT YOUNG, IF IT's NOT THE UKBSENVCJD ONLY STRAIN, YOUR OUTA LUCK.

YOUR SPORADIC, YOUR SPONTANEOUS, in short, your expendable. ...


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&P=1763


SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html


There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.

He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535


BRITISH MEDICAL JOURNAL

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406


BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#6117


JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535


doi:10.1016/S1473-3099(03)00715-1
Copyright © 2003 Published by Elsevier Ltd.
Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.


Volume 3, Issue 8, August 2003, Page 463


“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.”
............................


http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext

http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518




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