PRION PROTEIN AND THE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

Transmissible spongiform encephalopathies (TSEs) are fatal neurodegene rative diseases that occur in a wine variety of mammals. In humans, TS E diseases include kuru, sporadic and iatrogenic Creutzfeldt-Jakob dis ease (CJD) Gerstmann-Straussler-Scheinker syndrome (GSS) and fatal fam ilial insomnia (FFI). So far, TSE diseases occur only rarely in humans ; however, scrapie is a widespread problem in sheep, and the recent ep idemic of bovine spongiform encephalopathy (BSE or mad cow disease) ha s seriously affected the British cattle industry. Of special concern i s the recent appearance of a new variant of CJD in humans that is susp ected of being caused by infections from BSE-infected cattle products. In all these diseases, an abnormal form of a host protein, prion prot ein (PrP) is essential for the pathogenic process. The relationship of this protein to the transmissible agent is currently the subject of g reat interest and controversy and is the subject of this review.