Prions. David W. Colby 1 , * and Stanley B. Prusiner 1 , 2. Cellular Biology of Prion Diseases. Home.
TSEs in Humans. CJD Surveillance. Prions Abstract. Abstract Prions are unprecedented infectious pathogens that cause a group of invariably fatal neurodegenerative diseases by an entirely novel mechanism.
Prion diseases may present as genetic, infectious, or sporadic disorders, all of which involve modification of the prion protein (PrP). Bovine spongiform encephalopathy (BSE), scrapie of sheep, and Creutzfeldt–Jakob disease (CJD) of humans are among the most notable prion diseases. Prions are transmissible particles that are devoid of nucleic acid and seem to be composed exclusively of a modified protein (PrPSc).