Prion Diseases

The modern history of the prion diseases is one of novel microbes, anthropological intrigue, and food safety mishaps. The prion diseases, also called the transmissible spongiform encephalopathies, are fatal neurodegenerative diseases that can be sporadic, inherited, or acquired. These multiple origins are unique among human disease. The basis of all prion diseases is the misfolding of the host prion protein into the disease-specific prion protein conformation, called PrPSc. Transmission of PrPSc into naïve hosts can lead to additional prion protein misfolding and induction of neurodegenerative disease. Hence, prion diseases are transmissible but are caused by a novel pathogen that lacks a prion-specific nucleic acid genome.