Prions and Prion-like Proteins [Molecular Bases of Disease]

May 23rd, 2014 by Fraser, P. E.

Prions are self-replicating protein aggregates and are the primary causative factor in a number of neurological diseases in mammals. The prion protein (PrP) undergoes a conformational transformation leading to aggregation into an infectious cellular pathogen. Prion-like protein spreading and transmission of aggregates between cells has also been demonstrated for other proteins associated with Alzheimer's and Parkinson's disease. This protein-only phenomenon may therefore have broader implications in neurodegenerative disorders. The mini-reviews in this thematic series highlight the recent advances in prion biology and the role these unique proteins play in disease.