Prion diseases, also known as Transmissible Spongiform Encephalopathies (TSEs), exist in both humans (Creutzfeldt-Jakob disease (CJD)) and animals (scrapie, bovine spongiform encephalopathy (BSE), chronic wasting disease) and have the unique property of being infectious, sporadic or genetic in origin.

Although the precise nature of the infectious agent responsible for TSEs is not definitely identified, it is now clearly demonstrated that a protein named PrP (for Prion Protein) plays a critical role in the transmission and pathogenesis of TSEs.

This book provides the general...