Prions and prion diseases

Abstract

Prion diseases (transmissible encephalopathies) are progressive neurodegenerative diseases of animals and humans caused by a unique agent, the prion. These diseases have recently been in the news because of the emergence of bovine spongiform encephalopathy (BSE; mad cow disease) and variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom. Priori diseases may be sporadic, genetic, or acquired. Transmission results from abnormally folded proteins rather than nucleic acids. This article briefly reviews the molecular biology and biochemistry of the prion agent, the prion diseases, the evidence linking BSE to vCJD disease, and protocols for the handling and decontamination of prion-infected materials.