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A prion (pronounced /ˈpriː.ɒn/ ( listen)) is an infectious agent that is composed primarily of...

A prion (pronounced /ˈpriː.ɒn/ ( listen)) is an infectious agent that is composed primarily of protein. To date, all such agents that have been discovered propagate by transmitting a mis-folded protein state; the protein itself does not self-replicate and the process is dependent on the presence of the polypeptide in the host organism. The mis-folded form of the prion protein has been implicated in a number of diseases in a variety of mammals, including bovine spongiform encephalopathy (BSE, also known as "mad cow disease") in cattle and Creutzfeldt-Jakob disease (CJD) in humans. All known prion diseases affect the structure of the brain or other neural tissue, and all are currently untreatable and are always fatal. In general usage, prion refers to the theoretical unit of infection. In scientific notation, PrP refers to the endogenous form of prion protein (PrP), which is found in a multitude of tissues, while PrP refers to the misfolded form of PrP, that is responsible for the formation of amyloid plaques and neurodegeneration. Prions are hypothesized to infect and propagate by refolding abnormally into a structure which is able to convert normal molecules of the protein into the