Résumé
Transmissible spongiform encephalopathies, or prion diseases, including Creutzfeldt‐Jakob disease and Gerstmann‐Strässler syndrome in humans, and the scrapie and bovine spongiform encephalopathy in animals, are fatal neurodegenerative disorders. Human transmissible spongiform encephalopathies are unique in that they occur in infectious, sporadic or genetic forms. Although the nature of the infective agents, termed ‘prions’ is not fully understood, the conversion of the normal cellular prion protein, PrP
c
, to an abnormal protease‐resistant isoform PrP
Sc
, is a key event in the pathogenesis of all transmissible spongiform encephalopathies.