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prion diseases |
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prion diseases [′Prī‚än di‚zēz·əz] (medicine) A group of invariably fatal disorders affecting humans and animals that are clinically characterized by neurological and behavioral degeneration caused by the cerebral accumulation of an abnormal prion protein which is resistant to proteolytic enzymes and, in contrast to other infectious agents, does not require nucleic acid for replication. The diseases are transmissible either genetically (for example, Creutzfeldt-Jakob disease) or via infection (new variant Creutzfeldt-Jakob disease and mad cow disease) or can occur spontaneously (classical or sporadic Creutzfeldt-Jakob disease). Also known as spongiform encephalopathies. How to thank TFD for its existence? Tell a friend about us, add a link to this page, add the site to iGoogle, or visit webmaster's page for free fun content. |
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Transmissible subacute spongiform encephalopathies: prion diseases, Paris: Elsevier; 1996. Prion diseases can be inherited, as in fatal familial insomnia (the Italian family that couldn't sleep): they include other fatal neurological disorders such as Mad Cow and scrapies. However, identifying patients in early stages of prion diseases might be difficult, says Soto. |
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