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 ?
Clinical neurologic signs were observed on a few occasions without pathologic confirmation of prion disease, suggesting a nontransmissible spongiform encephalopathy condition related to the age of the mice in the study.
Kuru is a prion disease of humans that is of particular interest because of its historical place in our understanding of this unique constellation of diseases.
Moreover, the analysis of a broader and longer range of impacts extending well beyond the agricultural sector will promote the inclusion of societal values and ethics reflecting public concerns about prion disease risks.
Prion disease in people is manifested primarily by shaking.
National Institute of Allergy and Infectious Diseases and their colleagues in France and the United Kingdom have demonstrated that adding a peptide derived from the core of the normal prion protein dramatically reduced the generation of abnormal PrP associated with prion disease in rodent species (Chabry et al.
CJD accounts for more than 85% of all cases of prion disease.
I think feed or some other stuff was contaminated during that period,'' said Morikazu Shinagawa, head of the Prion Disease Research Center at the National Institute of Animal Health.
The National Agricultural Research Organization said it will set up the prion disease research center Oct.
Instead, the editor has assembled a cross-section of work by some of the leading researchers in the field and, to use his own words, "I have concentrated on the molecular pathogenesis of prion disease and the emphasis is on the role of the prion protein.
As the prion disease is continually passed in the new species, the incubation becomes shorter, eventually stabilizing.
Genetics still plays a part, because various mutations in the prion protein gene promote this transformation, while some polymorphisms (substitution of one base in the gene sequence for another) make individuals more susceptible to developing a prion disease.
In 2008 and 2010, we introduced a novel human prion disease, presumably sporadic, that we named variably protease-sensitive prionopathy (VPSPr) (14-16).