• If you are having problems logging in please use the Contact Us in the lower right hand corner of the forum page for assistance.

Beta-Amyloid and Pathological Prion Protein in sCJD

flounder

Well-known member
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)


--------------------------------------------------------------------------------

Key Words

Sporadic Creutzfeldt-Jakob disease
Alzheimer's disease
Deposition of -amyloid
Prion protein

--------------------------------------------------------------------------------

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


--------------------------------------------------------------------------------

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
 

PORKER

Well-known member
Abstract
The bovine spongiform encephalopathy (BSE) agent has been transmitted to humans, leading to variant Creutzfeldt-Jakob disease. Sheep and goats can be experimentally infected by BSE and have been potentially exposed to natural BSE; however, whether BSE can be transmitted to small ruminants is not known. Based on the particular biochemical properties of the abnormal prion protein (PrPsc) associated with BSE, and particularly the increased degradation induced by proteinase K in the N terminal part of PrPsc, we have developed a rapid ELISA designed to distinguish BSE from other scrapie strains. This assay clearly discriminates experimental ovine BSE from other scrapie strains and was used to screen 260 transmissible spongiform encephalopathy (TSE)–infected small ruminant samples identified by the French active surveillance network (2002/2003). In this context, this test has helped to identify the first case of natural BSE in a goat and can be used to classify TSE isolates based on the proteinase K sensitivity of PrPsc.

Transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases affecting humans and animals; examples are scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle, and chronic wasting disease in cervids. Strong evidence indicates that the BSE agent has been transmitted to humans through the food chain, leading to the emergence of variant Creutzfeldt-Jakob disease (vCJD). Recently, the first case of natural BSE was identified in a French goat (1), and a second probable one was reported in the United Kingdom (2). It seems, however, that these were isolated cases and that the BSE level in small ruminant flocks is probably low. However, BSE can be transmitted experimentally to sheep and goats by the oral route (3–5), whereas small ruminants have very likely been exposed to the BSE agent through contaminated meat and bone meal (MBM). Given the widespread tissue distribution of the BSE agent in sheep and goats, horizontal and vertical transmission within sheep and goat populations is possible, which could lead to human exposure to the agent through the food chain, even after the MBM feed ban.

TSEs are characterized by the accumulation in the brain of an abnormal form of the prion protein (PrPsc), which is derived from the normal one (PrPc) and manifests as unusual biochemical properties including insolubility in the presence of detergents and partial resistance to the action of proteases like proteinase K (PK). The conventional method of identifying a TSE strain is the experimental transmission of the disease in several mouse lines (6,7) including transgenic mouse lines that over express PrP, thereby increasing the efficiency and speed of transmission (8–10). However, these methods cannot be used as screening methods.
 

flounder

Well-known member
Saturday, March 22, 2008
10 Million Baby Boomers to have Alzheimer's in the coming decades
2008 Alzheimer?s disease facts and figures

Alzheimer?s Association*

Abstract Alzheimer?s disease is the seventh leading cause of all deaths in
the United States and the fifth leading cause of death in Americans older
than the age of 65 years. More than 5 million Americans are estimated to
have Alzheimer?s disease. Every 71 seconds someone in America develops
Alzheimer?s disease; by 2050 it is expected to occur every 33 seconds.
During the coming decades, baby boomers are projected to add 10 million
people to these numbers. By 2050, the incidence of Alzheimer?s disease is
expected to approach nearly a million people per year, with a total
estimated prevalence of 11 to 16 million persons. Significant cost
implications related to Alzheimer?s disease and other dementias include an
estimated $148 billion annually in direct (Medicare/Medicaid) and indirect
(eg, caregiver lost wages and out-of-pocket expenses, decreased business
productivity) costs. Not included in these figures are the estimated 10
million caregivers who annually provide $89 billion in unpaid services to
individuals with Alzheimer?s disease. This report provides information to
increase understanding of the public health impact of Alzheimer?s disease,
including incidence and prevalence, mortality, lifetime risks, costs, and
impact on family caregivers.


snip..


7.2. Impact of future reductions in deaths from other causes
As discussed in the Mortality section, the number of
deaths due to heart disease, cancer, and stroke, the three
leading causes of death, is decreasing. As a result, unless
there are new treatments to prevent Alzheimer?s and other
dementias, the remaining lifetime risk of Alzheimer?s and
dementia will increase substantially in the future because
the decreasing number of deaths from heart disease, cancer,
and stroke means that more people live long enough to
develop Alzheimer?s and other dementias.

7.3. Implications for baby boomers

The baby boomers are people living in the United States
now who were born from 1946 through 1964. In 2008, the
oldest baby boomers, people born in 1946, will be 62. The
youngest baby boomers, people who were born in 1964, will
be 44. The remaining lifetime risks of Alzheimer?s disease and
dementia shown in Figs. 9 and 10 apply to baby boomers
who are already age 55 or older. The remaining lifetime
risks of Alzheimer?s and other dementias also apply to baby
boomers who are younger than 55, assuming that they live
to be at least 55. The baby boomer group now includes about
78 million Americans, of whom 27 million are ages 55 to 62 and 51
million ages 44 to 54. Applying the proportions in Figs. 9
and 10, the Alzheimer?s Association estimates that 10 million
of these baby boomers can expect to develop Alzheimer?s
disease in their remaining lifetime (Appendix 16).
Similarly, about 14 million baby boomers can expect to
develop dementia, including Alzheimer?s disease.
As noted earlier, these figures are conservative because
of the relatively high threshold for including an individual
as a person with dementia in the Framingham Heart Study,
which is the source of the lifetime risk estimates (Appendix
16). True lifetime risk for baby boomers will probably be
greater for this reason. True lifetime risk for baby boomers
will also be greater because deaths from heart disease,
cancer, and stroke will probably continue to drop, increasing
the life span during which the boomers could develop
Alzheimer?s or other dementias.

References

snip... see full text 24 pages ;

© 2008 The Alzheimer?s Association. All rights reserved.


http://www.alzheimersanddementia.org/webfiles/images/journals/jalz/JALZ_739.pdf


Friday, March 21, 2008

Association between Deposition of Beta-Amyloid and Pathological Prion
Protein in Sporadic Creutzfeldt-Jakob Disease

http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=000121389


see full text Alzheimer's and CJD i.e. TSE, aka mad cow disease

http://betaamyloidcjd.blogspot.com/

http://betaamyloidcjd.blogspot.com/2008/03/association-between-deposition-of-beta.html


TSS
 

Latest posts

Top