Molecular and chemical basis of prion-related diseases

Abstract

Prion-related diseases include scrapie in sheep, bovine spongiform encephalopathy in cattle and Creutzfeldt-Jakob disease in humans. The infectious agent for these diseases surprisingly contains no nucleic acid, but is a protein(PrP) which exists in two conformations, PrP^C and PrP^Sc. The infectious PrP^Sc form has a higher β-sheet and lower α-helix content than PrP^C. The structures of PrP and models for how PrP^Sc is able to replicate by converting PrP^C to PrP^Sc are discussed.