Prions

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 spectrum of ‘prion-like’ diseases.

Key Concepts

• An overview of prion diseases that affect humans and animals.
• Biology of the cellular prion protein.
• Prion protein misfolding and disease.
• Proving the ‘protein-only’ hypothesis.
• The molecular basis of the species barrier and prion strains.
• An introduction to ‘prion-like’ protein aggregation.