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.

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 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. Also called subacute spongiform encephalopathy, transmissible neurodegenerative disease, transmissible spongiform encephalopathy.

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.

prion disease

See PRION PROTEIN, PRION PROTEIN DISEASE and CREUTZFELDT-JAKOB DISEASE.
References in periodicals archive ?
Many people who carry those variants never develop prion diseases.
Note that ME7 prion disease also generated a decline between the 17th and 18th wpi but this decline seems not to be related to viral infection.
Prion diseases are transmissible neurodegenerative disorders with different etiologies as they can be sporadic, genetic or acquired by infection (1,2).
Familial fatal insomnia (FFI) is a rare human prion disease caused by the D178N point mutation of the Prnp polymorphism M129V.
It is also incumbent on physicians to make the correct diagnosis so that transmission of this prion disease is avoided--particularly transmission via contaminated surgical equipment or medical procedures, they added.
There are good reasons for believing this response, identified with prion disease, applies also to Alzheimer's and other neurodegenerative diseases," he said.
CDC continues to conduct surveillance for cases of CJD in the United States through various mechanisms, including 1) receipt and investigation, in collaboration with local and state health departments, of case reports from physicians and patient support groups; 2) analysis of national multiple cause-of-death data; and 3) review of prion disease cases confirmed by the National Prion Disease Pathology Surveillance Center (NPDPSC) at Case Western Reserve University (Cleveland, Ohio).
In the long term, the project aims to add value to the research that others are conducting on prion disease risk management and identify policy and practice interventions for local, regional and national audiences.
Max, who himself has a neuromuscular disorder loosely related to the diseases he describes, wants to make a few political points about the relationship between prion diseases and government agricultural policies.
The study, published in the journal's June 24 edition, was based on the only example of a major human prion disease epidemic available--a disease called kuru that reached epidemic proportions in certain communities in Papua New Guinea and was caused by the practice of cannibalism, which ended in the 1950s.
Variant Creutzfeldt-Jakob disease could have an incubation time of 30 years or longer and could affect a wider population than currently forecast, according to new research on patients with a similar human prion disease.