Prions? CJD/BSE Connection...

brad nkeele at
Fri Mar 29 09:11:09 EST 1996

Sorry for the mishap on the first go -- I'll have another try:

Its been a few years since I've studied prion diseases, so my post may be a 
bit out-dated (flame-bait? bring it on).  I believe the term "prion" was 
coined by Stanley Prusiner of UCSF in the early '80s.  Prion diseases 
include bovine spongiform encephalopathy (BSE), scrapie in sheep, and kuru 
in humans.  These are all believed to be contracted by ingestion of the 
"mutant" protein.  A decline in the number of cases of kuru (in New Guinea?) 
after refraining from ritualistic  cannabilism suggests that if cows are not 
fed infected food or protein supplements, BSE may also dissapear. Prion 
diseases, though, are a little different that your run-of-the-mill 
communicable disseases.  There is a genetic component to prion diseases, in 
that certain herediary disorders, e.g. CJD, are also associated with 
abnormal prion protein.  Generally, these inheirited forms of prion disease 
result from a point mutation in the gene that produces a gene product that 
is abnormally processed.  Normally, prion proteins are integral membrane 
proteins with abundent expression in brain.  Somehow (and this may be known 
now) the abnormal prion converts the normal and it is no longer able to 
insert into the cellular membrane, and thus collects in the cytoplasm and 
exerts its toxic effects.  The end result (on autopsy) is a "holey," or 
spongiform, pathology.

Oh yeah, and some refs:

Prusiner (1982) Science 216:136.
Prusiner (1991) Science 252:1575.

So this guy Prusiner is _da man_ on prion diseases.

Hope this helped

In article <4jcdtm$n71 at>, cogito at says...
>Just recently there was an article in the Wall Street Journal
>regarding the possible discovery of an infectious/genetic agent that
>was neither a bacterium nor a virus.  The article was framed around
>the possible connnection between CJD and "Mad Cow Disease."  I believe
>the article named the offending agent as a "prion."  Does anyone know
>about this?
>Very curious,
>Jeffrey N. Browndyke                    
>Louisiana State University      Email: cogito at       
>Department of Psychology        Fax: (504) 388-4125
>236 Audubon Hall                URL:     
>Baton Rouge, LA. 70803
>Neuropsychology Central -

N. Bradley Keele
Neuroscience Graduate Program
UTMB - Pharmacology J-31
Galveston, TX  77445-1031
Voice:  (409) 772-9604
FAX:    (409) 772-9642
    "Once in awhile you get shown the light
      In the strangest of places
      If you look at it right."
                      --JG (1942-1995)                             

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