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 and propagation of the misfolded form of the prion protein, or prion. This article will review the key clinical and pathological features of prion diseases affecting animals and humans and the characteristics of the normal and disease‐associated forms of the prion protein. It will further dem..