Prions? CJD/BSE Connection...

Lai, Ken klai at pbi.nrc.ca
Wed Apr 3 11:19:56 EST 1996



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From: Lai, Ken
To: BIOSCI-REQUEST
Subject: Re: Prions?  CJD/BSE Connection...
Date: Thursday, March 28, 1996 6:18PM

Dag Stenberg     MD PhD                    stenberg at cc.helsinki.fi(wrote):
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Date: Thursday, March 28, 1996 1:30PM


""In short, prions are proteins that are found on/in normal cells. Their
function is unknown. The disease-inducing prion protein is capable of
catalyzing the transformation of normal prion protein into a form that
is resistant to biological degradation, and will be deposited in tissue,
for instance in the brain, eventually leading to dysfunction, cellular
death, and death of the individual.
  Known prion diseases in man are, for example, CJD, kuru, fatal familial
insomnia, and Gerstmann-Straussler-Scheinker disease (GSS).
  The disease form of the prion protein may apparently arise because of
mutation of its gene, or it can be transferred from protein material
from another individual. Until recently, it was believed that transfer
between species is negligible. Nowadays, it is kown that pathogenic
prion protein can be transferred between different species of ruminants,
and probably from sheep to cows, that is, it is possible that the "Mad
cow disease", or BSE (bovine spongioform encephalopathy) may come from
cattle having been fed food containing sheep protein......

     I would like to know if this protein can be degraded, as normal 
enyzmatic proteins, by heat exposure??  Thus can well cooked meat be 
declared prion-free?

Ken Lai




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