Dollco

Abstract

P rion diseases are rare, lethal, untreatable degenerative brain disorders of humans and animals. They are linked to conversion of a specific brain protein from its normal form (PrP C ) into a misfolded, pathologic form (PrP Sc ) and are transmissible between individuals by a novel protein-based mechanism. 1 Because there is no convincing evidence for causation by a conventional microbial patho gen, and because even a minute quantity of exogenous material containing PrP Sc can initiate a cascade of PrP C conversion in a new host in a manner reminiscent of infection, the term "prion" was coined to denote the implicated proteinaceous infectious particle