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Prion Diseases

Prat Itharat pitharat at sas.upenn.edu
Thu Apr 4 21:49:31 EST 1996

>In article <4juhm4$j22 at dfw-ixnews5.ix.netcom.com>, sequitur at ix.netcom.com
>(Janet) wrote:

>> Fatal diseases (many of a particularly humiliating slowly degenerative
>> sort), both genetic and acquired?  Transmissable without the benefit of
>> a nucleic acid genome?  Yikes!  Especially if you've ever had a
>> transfusion, since these infectious proteins aren't destroyed by heat
>> or irradiation.  

>Both GSS (genetic) and CJD (sporadic) are >>very<< rare human diseases. 
>The only aquired human scrapie disease has been kuru, found only among the
>natives of Papua/New Guinea who used to practice ritualistic cannabalism. 

There is still some debate as to the direct method of transmission in this 
case.  Natives could have been afflicted by handling infectious body parts 
[ie, prions entering through broken skin] and not necessarily by digesting.

Evidence also suggest that CJD was acquired by infectious GH treatment
[now, recombinant treatment can be used], corneal transplants, and contaminated
neurosurgical equipment.

[If you meant direct transmission, sorry.]

>>>IF<< there is a link between BSE (mad cow disease) and CJD, it is most
>likely due to passage of infectious agents by direct ingestion of tissue. 
>Also remember that cows are fed offal that includes brain and other nervous
>tissue, not flank steaks.  Therefore, it makes little sense to worry about
>human blood transfusions as a source of prion infection.  Just trying to
>keep things on a rational level here.

Quite true, as prions are mainly found in the CNS.
>John Corradi                      |   The opinions expressed above are my 
>The Rockefeller University        |   own and do not represent...blah,
>corradj at rockvax.rockefeller.edu   |   blah, blah.

Prat Itharat
pitharat at sas.upenn.edu

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