In a message dated 5/29/00 12:07:05 PM Pacific Daylight Time,
ABlainey@aol.com writes:
> What happens in the case of Pryons, like the type that *cause* C.J.D
and
> B.S.E.
> Are these ignored because they far too different from the defective
> protiens that the Chaperones usually enconter ?. Does a Pryon differ
> sygnificantly from a protien ? or are they just a biological aboration
> with
> the ability to act as catalysts and mutate other protiens ?. I know its
> probably a stupid question but my knowledge of the subject is very
limited,
> but its of great interest to me.
*NOT* a stupid question, at all.
I can't make any general statement about prions. In
the case of Alzheimer's and scrapie, the defective proteins
produce a breakdown product which is very, very resistant
to proteolysis. The lysosome suck it up, but can't degrade
it. Somehow this accumulation of junk and frustrated
lysosomes eventually kills the cells. Then other cells come
in to fix the mess but they're no better than the first set,
so you get this ever-growing mess.
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