prion diseases


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Related to prion diseases: Gerstmann-Straussler-Scheinker syndrome

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.
References in periodicals archive ?
In contrast to virtually all other sporadic human prion diseases, in which PK-resistant Pr[P.sup.D] (resPr[P.sup.D]) electrophoretically separates into 3 major bands, VPSPr resPr[P.sup.D] characteristically separates into 5 bands.
Phenotypic variability in familial prion diseases due to the D178N mutation.
Over the years, other prion diseases have been identified in both humans and animals (Table 1).
However, the latency period is now known to be at least 30 years and because of the known potential for even longer latency periods for prion diseases, this outbreak is likely to continue.
Lyketsos, "Rapidly progressive dementias and the treatment of human prion diseases," Expert Opinion on Pharmacotherapy, vol.
Prion diseases cause brain damage that worsens rapidly over time and are invariably fatal.
In the case of those rugby players and others who may have more secretively consumed human meat in recent decades, it can't be known definitively whether they will be safe from the same kuru disease the Fore tribe experienced, as prion diseases have long incubation periods, sometimes even decades long.
Prion diseases or transmissible spongiform encephalopathies (TSEs) are fatal neurological disorders that include Creutzfeldt-Jakob disease (CJD) and kuru in humans, scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle, and chronic wasting disease (CWD) in cervids.
Prion diseases are fatal neurodegenerative diseases characterized by accumulation of prion misfolded (PrPsc) protein, gliosis, synaptic dysfunction, and, at late stages, neuronal loss [1-5].
Prion diseases, also known as transmissible spongiform encephalopathies, comprise a group of incurable fatal neurologic disorders caused by prions.