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	va1-sg19016.securesites.net

	version=3.1.8



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Creutzfeldt-Jacob disease [abbreviated previously as vCJD or CJD (new 



The definitions of the designations deaths, definite cases, probable 

vCJD cases, can be found by accessing the UK Department of Health 



Data on vCJD cases from any part of the world are now included in 

iatrogenic, familial, and GSS -- Gerstmann-Straussler-Scheinker 

disease) are included also when they have some relevance to the 

incidence and etiology of vCJD.



In addition, prion-related diseases of domesticated and free-living 

animals may also be included if relevant. - Mod.CP]





******

(CJD) Statistics [edited]





Monthly Creutzfeldt-Jakob disease statistics - 2 Oct 2007

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

The Department of Health is today [Mon 2 Oct 2007] issuing the latest 

information about the numbers of known cases of Creutzfeldt-Jakob 

disease (CJD). This includes cases of variant Creutzfeldt-Jakob 

form of the disease thought to be linked to BSE (bovine spongiform 

encephalopathy).



Definite and probable CJD cases in the UK as of 28 Sep 2007

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

Summary of vCJD cases -- deaths

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



Summary of vCJD cases -- alive

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



Total

-----



N.B. Following this press notice the Department of Health will stop 

issuing monthly CJD press notices because the same data are also 

published by the National CJD Surveillance Unit in Edinburgh. Up to 

the website of the National CJD Surveillance Unit at 



These data are still consistent with the view that the vCJD outbreak 

in the UK is in decline. The peak number of deaths was 28 in the year 

2000, followed by 20 in 2001, 17 in 2002, 18 in 2003, 9 in 2004, 5 in 

2005, 5 in 2006, and so far 3 in 2007.



Totals for all types of CJD cases in the UK since 1995

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

As of 28 Sep 2007, in the UK in the year 2007, so far there have been 

82 referrals, 30 deaths from sporadic CJD, 2 deaths from iatrogenic 

CJD, 2 deaths from familial CJD, one from GSS, and 3 deaths from vCJD.



--



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Alberta public release [edited]





Scientists have discovered a new protein that may offer fresh 

insights into brain function in mad cow disease. "Our team has 

defined a 2nd prion protein called 'Shadoo', that exists in addition 

to the well-known prion protein called 'PrP'," said Professor David 

Westaway, director of the Centre for Prions and Protein Folding 

Diseases at the University of Alberta.



"For decades we believed PrP was a unique nerve protein that folded 

view is no longer accurate," Westaway adds.



The study was conducted jointly by the University of Toronto, 

University of Alberta, Case Western Reserve University (Ohio), and 

the McLaughlin Research Institute (Montana). The research is 

published today [16 Aug 2007] in the EMBO (European Molecular Biology 

Organization) Journal and represents a culmination of work initiated 

at the University of Toronto in 1999, and then continued more 

recently at the University of Alberta.



This is the 1st discovery since 1985 of a new brain prion protein. "A 

2nd prion protein had been inferred by other research, based on 

indirect studies and the examination of DNA sequences," said lead 

author Joel Watts, a graduate student at the University of Toronto's 

Centre for Research in Neurodegenerative Diseases. "But we not only 

demonstrate that this theoretical protein really exists and shares 

several properties with healthy PrP; we have also defined an 

unexpected alteration in prion infections.



"As the PrP molecule alters shape and accumulates in a prion-affected 

brain, the Shadoo protein seems to disappear," Watts added. Since 

proteins in a living cell are the molecules "that do the work, this 

is likely to be significant," he said.



"Many facets of a prion disease like BSE are puzzling," Westaway 

said. "The puzzles include the cause of death of brain cells, the 

function of normal prion proteins, and the rules governing emergence 

and spread of prions from animal to animal. We believe the Shadoo 

protein can give us a fresh purchase on these important questions."



--

Terry S. Singeltary Sr.





[The EMBO Journal advance online publication appeared on 16 Aug 2007; 



"The CNS glycoprotein Shadoo has PrP/C-like protective properties and 

displays reduced levels in prion infections

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



Authors

-------

Joel C Watts (1, 2), Bettina Drisaldi (1), Vivian Ng (1), Jing Yang 

(1), Bob Strome (1), Patrick Horne (1), Man-Sun Sy (3), Larry Yoong 

(1), Rebecca Young (4), Peter Mastrangelo (1), Catherine Bergeron (1, 

2), Paul E Fraser (1, 5), George A Carlson (4), Howard T J Mount (1, 

6), Gerold Schmitt-Ulms (1, 2), and David Westaway (1, 2, 7)



1. Centre for Research in Neurodegenerative Diseases, University of 

Toronto, Toronto, Canada

2. Department of Laboratory Medicine and Pathobiology, University of 

Toronto, Toronto, Canada

3. Department of Pathology, School of Medicine, Case Western Reserve 

University, Cleveland, OH, USA

4. McLaughlin Research Institute, Great Falls, MT, USA

5. Department of Medical Biophysics, University of Toronto, Toronto, Canada

6. Department of Medicine, University of Toronto, Toronto, Canada

7. Centre for Prions and Protein Folding Diseases, University of 

Alberta, Alberta, Canada



Abstract

--------

The cellular prion protein, PrP/C, is neuroprotective in a number of 

settings and in particular prevents cerebellar degeneration mediated 

by CNS (central nervous system)-expressed Doppel or internally 

deleted PrP ('delta PrP'). This paradigm has facilitated mapping of 

activity determinants in PrP/C and implicated a cryptic PrP/C-like 

protein ['pi']. Shadoo (Sho) is a hypothetical GPI 

(glycosylphosphatidylinisotol)-anchored protein encoded by the _Sprn_ 

gene, exhibiting homology and domain organization similar to the 

N-terminus of PrP. Here we demonstrate _Sprn_ expression and Sho 

protein in the adult CNS. Sho expression overlaps PrP/C, but is low 

in cerebellar granular neurons (CGNs) containing PrP/C and high in 

PrP/C-deficient dendritic processes. In _Prnp_0/0 CGNs, Sho 

transgenes were PrP/C-like in their ability to counteract neurotoxic 

effects of either Doppel or delta-PrP. Additionally, prion-infected 

mice exhibit a dramatic reduction in endogenous Sho protein. Sho is a 

neuroprotective activity, compromised neuroprotective activity 

resulting from reduced levels may exacerbate damage in prion 

infections. Sho may prove useful in deciphering several unresolved 

facets of prion biology." - Mod.CP]



******





Taiwan CDC clarifies media report of Creutzfeldt-Jakob disease (CJD) 

transmission through placenta extract injection

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

On 30 Aug 2007, a print medium in Taiwan reported that "Placenta 

extracts could lead to Creutzfeldt-Jakob disease (CJD)" [see 

reference to this report, Taiwan CDC (Centers for Disease Control) 

states that the Creutzfeldt-Jakob Disease Advisory Committee held a 

meeting this March [2007] after a suspected CJD case was reported by 

National Cheng-Kung University (NCKU) Hospital.



Based on the limited information provided by NCKU Hospital, the 

patient's neurological characteristics and symptoms did not fully 

meet the case definition of CJD. Hence, the committee has requested 

the hospital to provide more detailed information about the patient 

and has also scheduled another meeting to further investigate the case.



Another print medium has also reported that the same patient may have 

used placenta extracts. However, an epidemiological investigation 

conducted by the public health personnel indicated that the patient 

has no record of administering placenta extract injection, which 

suggests that the report may be incorrect. Further, Taiwan CDC also 

adds that currently no incidences of CJD in humans that are caused by 

injection of placenta extracts have been reported yet in the world.



CJD is a rare and incurable degenerative neurological disorder. It is 

caused by the accumulation of prions in the nerve cells, which 

results in spongiform and many neurological symptoms. The traditional 

form of CJD is spontaneous and may be inherited from one's parents. 

On average, it affects people aged around 65. Although cases of 

human-to-human transmission have been reported, they were either 

caused by organ transplants or by blood transfusions. Thus far, no 

reports of CJD transmission between humans have been associated with 

body contact. On the other hand, another variant of CJD is called 

vCJD, which may be related to bovine spongiform encephalopathy (BSE).



The common name for BSE is mad cow disease. Approximately 200 cases 

of vCJD have been reported worldwide, mainly in Western Europe, 

including England, France, and Ireland. Since 1990, Taiwan has banned 

imports of live cattle (sheep) as well as their by-products that may 

contain living tissues such as meat and bone meal, meat meal, bone 

meal, blood meal, feeding oil, embryos, cosmetic products containing 

bovine or sheep tissues such as placenta extracts, and serums. In 

Taiwan, CJD patients and people who visited Western Europe during a 

specific time period are prohibited from donating blood.



Since 1997, the health authorities in Taiwan have been monitoring 

CJD. Until August 2007, 180 cases have been reported and confirmed in 

Taiwan. Over the decade, the yearly average incidence of CJD in 

Taiwan is 0.5-1 cases per million population, which is the same as 

the world average. Experts in this domain have also confirmed that 

all confirmed cases in Taiwan are traditional CJD and no cases of 

vCJD have been observed in Taiwan. Nevertheless, Taiwan CDC will 

continue to monitor the occurrence of this disease to ensure the 

health of people in Taiwan.



For more information on CJD and the related preventive measures, 

consultation hotline 1922 operated by Taiwan CDC. The information 

provided by Taiwan CDC can prevent the transmission of CJD and avoid 

unnecessary panic due to lack of knowledge about the disease.



--



2006

----

CJD (new var.), blood transfusion risk 20061208.3468

CJD, transmission risk - Canada (ON) 20061207.3457

2005

----

2004

----

CJD, genetic susceptibility 20041112.3064

CJD (new var.), blood supply - UK 20040318.0758

CJD (new var.), carrier frequency study - UK 20040521.1365

2003

----

2002

----

2001

----

CJD (new var.), incidence & trends - UK (02) 20011124.2875

CJD (new var.), incidence & trends - UK 20011115.2816

...................................cp/mj/mpp