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 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 demonstrate how scientific research has contributed to our understanding of how a misfolded protein can transmit disease between individuals of the same and different species.

Key Concepts

• Prion protein misfolding and disease.
• An overview of prion diseases that affect humans and animals.
• Biology of the cellular prion protein.
• Proving that a protein can transmit disease.
• What is the species barrier and prion strains.
• An introduction to ‘prion-like’ protein aggregation.