Is a prion ...

Karl Roberts kr1 at PGSTUMAIL.PG.CC.MD.US
Thu May 15 14:26:21 EST 1997

Thank you David.  Just what I wanted to hear.

On 15 May 1997, Enigl wrote:

> In article <337A5809.6298D92E at utoronto.ca>, Martin Jugenburg
> <martin.jugenburg at utoronto.ca> writes:
> >
> >Prion is a regular brain protein that just happens to be modified
> >(glycosylated) in a unique manner. 
> I've seen this error before.  It's time I said something to correct the
> misconception, i.e. the _incorrect_ use of the word prion.  A prion is the
> _abnormal_ form (PrPsc) NOT the normal form of the glycoprotein PrPc.  A
> prion should not be confused with a "regular" brain protein PrPc.  The
> prion is a lethal, pathological, infectious agent, protein.  
> Theory:  A prion post-translationally combines with the normal protein
> PrPc to form a heterodimer (PrPc/PrPsc) then to a homodimer (PrPsc/PrPsc).
>  The gene for the normal protein is on chromosome 20.  The toxic form is
> made before PrPc is sialyated within the Golgi apparatus and before entry
> into lysosomes.  PrPsc accumulated in the vacuoles and secondary lysosomes
> leading to cell death (highest concentration in the neurons).  
> The function of the normal ( _NON-prion_ ) PrPc is unknown.  
> Davin
> Davin C. Enigl, MEAS (Master of Environmental Arts and Sciences), S.P.
> President-Microbiologist
> Microbiology Consulting,  Hazard Analysis and
> Critical Control Points (HACCP), CGMP, and Validations
> for the Food, Cosmetic, Nutritional  Supplement, and Pharmaceutical
> Industry
> enigl at aol.com
> http://members.aol.com/enigl/index.html
> May 15, 1997
> 8:05 am PACIFIC

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