Prions

Abstract

Abstract Prion diseases are invariably fatal neurodegenerative disorders associated with the aberrant folding of the normal cellular prion protein. The disease affects both humans and animals and in humans occurs in sporadic, familial and acquired forms. In the absence of a conventional infectious agent, the acquired forms of the disease occur through the transmission of the misfolded form of the prion protein, or prion. This article highlights the evidence for the principle of a ‘protein‐only’ transmissible disease and addresses some of the controversies including the existence of prion strains and the species barrier. It will also consider the expanding ..