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A prion ([1]) — short for proteinaceous infectious particle (-on) that lacks nucleic acid (by analogy to virion) — is a type of infectious agent composed only of protein. They cause a number of diseases in a variety of animals, including bovine spongiform encephalopathy (BSE, also known as mad cow disease) in cattle and Creutzfeldt-Jakob disease in humans. All prion diseases affect the structure of the brain or other neural tissue, and all are untreatable and fatal.