Prions are abnormal proteins that attack the brain from inside. None of the human prion diseases are common and animal prion diseases are more common. The various transmissible spongiform encephalopathies in animals caused by prions include the famous "mad cow disease" (BSE), scrapies in sheep, chronic wasting disease of deer and elk, and various others. Human prion diseases include CJD, variant CJD (possibly linked to BSE), the obscure kuru (from brain cannibalism in New Guinea), Gerstmann-Straussler-Scheinker disease, and the bizarre fatal familial insomnia (where a person literally dies because they cannot fall asleep). See detailed discussion in article Prion Diseases.
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Small proteinaceous infectious particles which resist inactivation by procedures that modify NUCLEIC ACIDS and contain an abnormal isoform of a cellular protein which is a major and necessary component. The abnormal (scrapie) isoform is PrPSc (PRPSC PROTEINS) and the cellular isoform PrPC (PRPC PROTEINS). The primary amino acid sequence of the two isoforms is identical. Human diseases caused by prions include CREUTZFELDT-JAKOB SYNDROME; GERSTMANN-STRAUSSLER SYNDROME; and INSOMNIA, FATAL FAMILIAL. - (Source - Diseases Database)
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