Original Paper
Association between Deposition of Beta-Amyloid and Pathological Prion Protein in Sporadic Creutzfeldt-Jakob Disease
Laura Debatina, Johannes Strefferb, Markus Geissenc, Jakob Matschkec, Adriano Aguzzia, Markus Glatzela, c
aInstitute of Neuropathology, and
bDivision of Psychiatry Research, University Hospital Zurich, Zurich, Switzerland;
cInstitute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
Address of Corresponding Author
Neurodegenerative Dis (DOI: 10.1159/000121389)
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Key Words
Sporadic Creutzfeldt-Jakob disease
Alzheimer's disease
Deposition of -amyloid
Prion protein
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Abstract
Background: Alzheimer's disease (AD) and prion diseases such as sporadic Creutzfeldt-Jakob disease (sCJD) share common features concerning their molecular pathogenesis and neuropathological presentation and the coexistence of AD and CJD in patients suggest an association between the deposition of the proteolytically processed form of the amyloid precursor protein, -amyloid (A), which deposits in AD, and the abnormal form of the prion protein, PrPSc, which deposits in sCJD. Methods: We have characterized sCJD patients (n = 14), AD patients (n = 5) and nondemented controls (n = 5) with respect to the deposition of PrPSc and A morphologically, biochemically and genetically and correlated these findings to clinical data. Results: sCJD-diseased individuals with abundant deposits of A present with a specific clinicopathological profile, defined by higher age at disease onset, long disease duration, a genetic profile and only minimal amounts of PrPSc in the cerebellum. Conclusion: The co-occurrence of pathological changes typical for sCJD and AD in combination with the inverse association between accumulation of A and PrPSc in a subgroup of sCJD patients is indicative of common pathways involved in the generation or clearance of A and PrPSc in a subgroup of sCJD patients.
Copyright © 2008 S. Karger AG, Basel
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Author Contacts
Markus Glatzel
Institute of Neuropathology, University Medical Center Hamburg-Eppendorf
Martinistrasse 52, DE-20246 Hamburg (Germany)
Tel. +49 40 42 803 2218, Fax +49 40 42 803 4929
E-Mail
[email protected]
http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=000121389
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Since this article does not have an abstract, we have provided the first 150
words of the full text and any section headings.
To the Editor:
In their Research Letter, Dr Gibbons and colleagues1 reported that the
annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable
since 1985. These estimates, however, are based only on reported cases, and
do not include misdiagnosed or preclinical cases. It seems to me that
misdiagnosis alone would drastically change these figures. An unknown number
of persons with a diagnosis of Alzheimer disease in fact may have CJD,
although only a small number of these patients receive the postmortem
examination necessary to make this diagnosis. Furthermore, only a few states
have made CJD reportable. Human and animal transmissible spongiform
encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr
Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob
disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL
TEXT
http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT
JOURNAL OF NEUROLOGY
MARCH 26, 2003
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
Regarding Alzheimer's disease
(note the substantial increase on a yearly basis)
http://www.bseinquiry.gov.uk/files/yb/1988/07/08014001.pdf
snip...
The pathogenesis of these diseases was compared to Alzheimer's disease at a molecular level...
snip...
http://www.bseinquiry.gov.uk/files/yb/1990/03/12003001.pdf
And NONE of this is relevant to BSE?
There is also the matter whether the spectrum of ''prion disease'' is wider than that recognized at present.
http://www.bseinquiry.gov.uk/files/yb/1990/07/06005001.pdf
Human BSE
snip...
These are not relevant to any possible human hazard from BSE nor to the much more common dementia, Alzheimers.
snip...
http://www.bseinquiry.gov.uk/files/yb/1990/07/09001001.pdf
IN STRICT CONFIDENCE
TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES
http://www.bseinquiry.gov.uk/files/yb/1993/01/05004001.pdf
Subject: Re: Hello Dr. Manuelidis Date: Fri, 22 Dec 2000 17:47:09 -0500 From: laura manuelidis <
[email protected]> Reply-To:
[email protected] Organization: Yale Medical School To: "Terry S. Singeltary Sr." <
[email protected]>
References: <
Dear Terry,
One of our papers (in Alzheimer's Disease Related Disord. 3:100-109, 1989) in text cites 6 of 46 (13%) of clinical AD as CJD. There may be a later paper from another lab showing the same higher than expected incidence but I can't put my hands on it right now. We also have a lot of papers from 1985 on stating that there are likely many silent (non-clinical) CJD infections, i.e. much greater than the "tip of the iceberg" of long standing end-stage cases with clinical symptoms. Hope this helps.
best wishes for the new year laura manuelidis
"Terry S. Singeltary Sr." wrote: > > Hello again Dr. Manuelidis, > > could you please help me locate the 2 studies that were > done on CJD where it showed that up to 13% of the people > diagnosed as having Alzheimer's actually had CJD. > trying to find reference... > > thank you, > Terry S. Singeltary Sr.
==================================
http://neurotalk.psychcentral.com/thread13175.html
http://neurotalk.psychcentral.com/showthread.php?p=156015
TSE UPDATE (SEE LINKS BELOW)
Thursday, March 13, 2008
DOWNER COW BLUES SENATORS WANT CRACKDOWN
http://downercattle.blogspot.com/2008/03/downer-cow-blues-senators-want.html
Thursday, March 6, 2008
House committee subpoenas Hallmark/Westland CEO - i call for an
investigation of the investigators
http://downercattle.blogspot.com/2008/03/house-committee-subpoenas.html
Friday, March 7, 2008
QUESTIONS AND ANSWERS HALLMARK/WESTLAND MEAT PACKING CO.
March 6, 2008
Consumer Concerns
Q. My child/school recently consumed Hallmark/Westland products. What is the
risk to children's health?
SEE FULL TEXT ;
http://downercattle.blogspot.com/2008/03/usda-questions-and-answers.html
March 16, 2008
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or
Italian L-BASE
http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html
IN FY 2007 TWO FIELD CASES, ONE VALIDATION CASE, AND TWO RSSS CASES WERE
CONSISTENT WITH NOR-98 SCRAPIE. ...
(BRINGS A TOTAL OF 5 NOR-98 CASES DOCUMENTED IN 2007 IN USA. ...TSS)
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps
http://nor-98.blogspot.com/
http://scrapie-usa.blogspot.com/
CWD
http://chronic-wasting-disease.blogspot.com/
TME
http://transmissible-mink-encephalopathy.blogspot.com/
TSS