Molecular biology of prion diseases.

What is the nature of the transmissible agent responsible for neurodegenerative diseases such as scrapie and mad-cow disease in animals and Creutzfeldt-Jakob disease in man? There is now weighty evidence that PrP(Sc), a modified version of the ubiquitously expressed host protein PrP(C), is responsible for pathogenesis of these diseases and that conversion of PrP(C) into PrP(Sc) under the influence of PrP(Sc) is the process leading to the propagation of PrP(Sc) and disease progression.