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Keyword - Prion (KW-0640)

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Protein which is able to form a prion, a self-propagating protein conformation (infectious protein). Formation of prions involves conversion of the native proteins into an infectious, amyloid form. This class of proteins includes the members of the prion family which can form proteinaceous infectious particles responsible for transmissible spongiform encephalopathies (TSE) in many animals, such as Kuru and Creutzfeldt-Jakob syndrome in humans, scrapies in sheep and BSE in cattle. These prion diseases represent special cases of neurodegenerative amyloidoses, because they result from the transmissibility and infectivity of the amyloid.


Molecular function


PrionAmyloidMolecular functionCellular component
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