Cruetzfeldt-Jakob Disease and CJD Voice

Len Bell lgbell* at *liv.ac.uk
Wed Apr 15 04:14:58 EST 1998


James Mosby wrote:
> 
> Is it proven the CJD is caused by a prion?  It is my understanding that there is
> much opposition to this theory.  

Well Stanley Prusinger recieved a nobel prize for his work/ theory on
Prions and a brief search of MedLine would bring you a host of
references which make the association between CJD, BSE, Kuru, Scrapie
and several other conditions with the Prion hypothesis.

Certainly if CJD is caused by a more traditional vector, such as a virus
or bacteria, then forget Kelvar get a coat made out of Prion Protein (or
whatever it is that protects the nucleic acid core in the slow virus
hypothesis) and your be protected from almost anything- including hard
X-rays and high intensity UV!

>I know that "filamentous" bodies have been
> recovered from autopsies, 

These filamentous bodies, are IIRC, 'PrP Amyloid' which have a
simmilarity in structure (though not in the identity of the component
protein) to the Beta-Amyloid Plaques found in Alzhiemers disease
individuals.

> but I do not understand how an "inanimate" protein can
> induce the conversion of a normal protein to the prion state?  

If you did have a clear understanding and could prove your
interpretation, youd be on the way to scientific fame!

>If anyone can
> briefly explain this event, it would be appreciated.   Bo
> 

One explanation which has a certain appeal is that it is akin to a
crystalisation process.  Once, one PrP protein has adopted the 'Scrapie'
(Sc) conformation it becomes, less soluble and is resistant to normal
cell metabolism and breakdown by proteases, it also is unable to perform
whatever the actual biological function of normal PrP is.  This leads to
an up regulation in the production of normal PrP to compensate for this
loss of activity.  Once PrP (Sc) has started to be deposited it acts as
a seed for a 'crystalization' type, a sort of seeded aggregation,
process promoting the aggregation of native PrP into the PrP Sc form. 
It is this build up of PrP Sc plaques which is considered crucial to the
the progression of CJD.

I certainly found this analogy convenient when I was considering/
describing the depostion of Beta-Amyloid in my PhD on Alzheimers
disease.


Regards,
Len

> DebbieOney wrote:
> 
> > Creutzfeldt-Jakob Disease (CJD) is a horrendious fatal brain-deteriorating
> > disease for which there is no treatment or cure.  It is caused by a prion. One
> > strain of CJD is linked to Mad Cow Disease in England.  This strain has not
> > been found in North America.  In the United States people get CJD through 3
> > means: familial (genetic), spontaneous (don't know how) and iatrogenic
> > (through a medical procedure).  It can take decades after exposure for the
> > patient to show symptoms.
<snip>
> > Please feel free to visit our website and to refer other people to it.
> > Please have them contact Liz Armstrong at LArmstr853 at aol.com about getting on
> > the e-mail list.
> >
> > Thank you.
> >
> > Deborah Schechter

-- 
Dr Len Bell,
University of Liverpool.
email: lgbell at liv.ac.uk 

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