prion disease


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Related to prion disease: Creutzfeldt-Jakob disease

prion

 [pri´on]
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 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). Inherited forms result from mutations in the gene that codes for prion protein; such mutations may also occur sporadically. Hereditary forms include some forms of Creutzfeldt-Jakob disease, Gerstmann-Sträussler syndrome, and fatal familial insomnia. Infectious forms of the disease result from ingestion of infected tissue or the introduction of infected tissue into the body (kuru and some forms of Creutzfeldt-Jakob disease). The latter has occasionally occurred during surgical procedures; it has also occurred as the result of injection of human growth hormone prepared from infected pituitary glands. Prion diseases also occur in animals. Called also transmissible neurodegenerative disease and subacute spongiform or transmissible spongiform encephalopathy.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

prion disease

Any transmissible neurodegenerative disease believed to be caused by a proteinaceous infectious particle (also known as prion proteins, or PrPs). PrPs change other cellular proteins, producing intracellular vacuoles (“spongiform change”) that disrupt the functioning of neurons. Included in this group are Creutzfeldt-Jacob disease, Gerstmann-Strüssler-Scheinker syndrome, kuru, and fatal familial insomnia in humans, mad cow disease (bovine spongiform encephalopathy), and scrapie in sheep and goats. Prion diseases may be transmitted by hereditary changes in the gene coding PrP; by contaminated biological agents such as plasma or serum, human growth hormone, and organ transplants; and possibly, by eating the flesh of infected animals. All prion diseases are characterized by a long incubation period, followed by a rapidly progressive dementia.
Medical Dictionary, © 2009 Farlex and Partners

prion disease

See PRION PROTEIN, PRION PROTEIN DISEASE and CREUTZFELDT-JAKOB DISEASE.
Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005
References in periodicals archive ?
Prion disease tempo determined by host-dependent substrate reduction.
Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene.
Prion diseases are rare neurodegenerative disorders identified as transmissible spongiform enchephalopathies (TSEs).
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.
Property PMCA RT-QuIC Prion disease vCJD Sporadic, familial, specificity and acquired [PrP.sup.C] Transgenic mouse Truncated recombinant substrate brain homogenate hamster [PrP.sup.C] expressing human (2nd generation assays) [PrP.sup.C] Fragmentation Sonication Shaking technique Fluids and tissues Blood, urine CSF, nasal brushings tested Detection Western blot Thioflavin T fluorescence Turnaround time [congruent to]11 days <24 h Currently in No Yes clinical use
(2008) Sigle treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease. Proc.
Because we have not investigated possible environmental influence on aged mice prion disease progression, no data from aged mice raised on enriched cages are exhibited.
In biochemical laboratory settings, [PrP.sup.Sc], the most reliable marker of prion disease, can be identified on the basis of its protease-resistant properties [2].
The couple worked with Harvard University geneticist Daniel MacArthur to examine genetic data from 16,025 people with prion diseases and 60,706 others who had the protein-coding parts of their genomes sequenced as part of the ExAC project, an effort to find disease-causing genetic variants (SN: 12/12/15, p.
The National Prion Disease Pathology Surveillance Center (NPDPSC) in Cleveland, Ohio, USA, was contacted and agreed to fly to Puerto Rico to perform the autopsy.
Even so, as a physician I don't fret much about epidemics of prion diseases turning the population into brain-eating zombies.