A disease due to a prion, a proteinaceous infectious particle that lacks nucleic acids.
Definition of PRION. : any of several protein particles that are abnormal forms of normal cellular proteins, that lack nucleic acid, and that in mammals have been implicated as the cause of prion diseases when accumulated in the mammalian brain. prion disease any of a group of fatal degenerative diseases of the nervous system caused by abnormalities in the metabolism of prion protein. These diseases are unique in that they may be transmitted genetically as an autosomal dominant trait, or by infection with abnormal forms of the protein (prions). Several conditions fall under the term prion diseases. A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally. Prion diseases can affect both humans and animals and are sometimes transmitted to humans by infected meat products. prion. any of several pathogenic, transmissible forms of the core of prion protein that cause a group of degenerative diseases of the nervous system known as prion diseases. Prions have a structure different from that of normal prion protein, lack detectable nucleic acid, and do not elicit an immune response. Prion disease: A disease due to a prion, a proteinaceous infectious particle that lacks nucleic acids. Prions are composed largely, if not entirely, of an altered formal (an abnormal isoform) of a normal cellular protein. The known prion diseases of humans and other mammals are: 1  Bovine spongiform encephalopathy (BSE) -- also called mad cow disease; the natural hosts are cattle; the prion involved is the BSE prion. 2  Creutzfeldt-Jakob disease (CJD) -- the natural hosts are humans; the prion involved is the CJD prion. Definition of PRION DISEASE. : any of a group of spongiform encephalopathies that are caused by prions and that include bovine spongiform encephalopathy, Creutzfeldt-Jakob disease, kuru, scrapie, and variant Creutzfeldt-Jakob disease —called also transmissible spongiform encephalopathy. ADVERTISEMENT.
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prion disease definition