Cruetzfeld-Jacob Disease and CJD Voice

[Jay Mone']

James,
While many of the precise details still remain unknown, all of the 
epidemiological and molecular evidence supports the prion hypothesis.  
Many excellant reviews have been published over the past few years, 
the most recent being Prusiner, 1997, Science 278:245.
There are, in short, two theories:
(1)  template assistance: mixing of normal prion proteins with scrapie 
prions proteins results in the formation of hetrodimers, which 
dissociate, releasing new scrapie prions.
(2)  Nucleated polymerization: Similar to crystal seeding.

There is also the possibility of a chaperone protein which may 
participate in the  induced folding of normal prion proteins into 
abnormal ones, but this protein has yet to be identified.

Jay Mone'