spongiform encephalopathy in young humans

Thu Nov 9 14:42:07 EST 1995

John Brunstein (brunstei at UNIXG.UBC.CA) wrote:

: On 8 Nov 1995, A.J.Cann wrote:

: > mremingt at UMABNET.AB.UMD.EDU ("Mary P. Remington") wrote:
: > >Two "Letters to the Editor" appeared in the Oct. 28, 1995 Lancet.  CJD 
: > >was histologically confirmed in an 18 yr.old male in the UK without 
: > >family history of CJD.  A 16 yr.old girlwas also confirmed histologically 
: > >to have CJD.

: > 
: > Hmm... I wonder if these numbers are statistically significant?   ;-)

: ******  They are if you happen to be one of those two people.   ******

: > 
: > Dr Alan J. Cann  PhD,   Department of Microbiology & Immunology,
: > University of Leicester,  P.O. Box 138,  Medical Sciences Building,
: > University Road, Leicester LE1 9HN,  UK.
: > Email: nna at le.ac.uk     http://www-micro.msb.le.ac.uk/AJC/nna.html
: > 
: > 
: > 
: > 
I was wondering if it has been determined how the PrP (sc) aggravates a 
conformational change in the PrP (c) protein in the host brain?  My text 
says it forms into a heterodimer, and compells a cascading effect; and 
that there is no immune response.  But I would be curious to know exactly 
what chemical shift occurs to cause this (according to this it takes only 
one amino acid point mutation to change PrP (c) to PrP (sc)).


Matt Wettlaufer

I feel very sorry for those two individuals with CJD--you're right, 
it is statistically important to them.  It is a very tragic disease.

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