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Definition: prion from Philip's Encyclopedia

Infective agent that appears to consist simply of a protein. Prions are thought to cause diseases such as Creutzfeldt-Jakob disease (CJD) and kuru in humans, Bovine Spongiform Encephalopathy (BSE) in cattle, and scrapie in sheep. It is not yet understood how prions work; unlike viruses and bacteria, they do not contain DNA or RNA. A virus is a hundred times larger than a prion. The US neurologist Stanley Prusiner won the 1997 Nobel Prize for developing the prion theory in 1982.

prion

From The Hutchinson Unabridged Encyclopedia with Atlas and Weather Guide
Infectious agent, a hundred times smaller than a virus. Composed of protein, and without any detectable nucleic acid (genetic material), it is strongly linked to a number of fatal degenerative brain diseases in mammals, such as bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep, and Creutzfeldt–Jakob disease (CJD) and kuru in humans. The existence of prions was postulated by US neurologist Stanley Prusiner in 1982, when he and his colleagues isolated a single infectious agent for scrapie that consisted only of protein and had no associated nucleic acid (RNA or DNA). His theory has been upheld by subsequent research, which has identified the protein concerned as well as a mechanism for its action. A US researcher proved that prions are capable of spreading disease, in July 2000, three years after Prusiner was awarded the Nobel Prize for Physiology or Medicine for his prion theory. Prions have been found to consist of a simple protein called PrP, made up of about 250…
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Full text Article prion

From Dictionary of Microbiology & Molecular Biology
(1) In man/animals: an aberrant form of a normal, chromosome-encoded glycoprotein; prions are now believed to be the aetiological agents of TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES . [Mapping the parameters of prion-induced neuropathology: PNAS (2000) 97 10573–10577.] The normal protein, which is…
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Full text Article prion

From The Columbia Encyclopedia
(prē'ŏn), abnormal form of a protein found in mammals, believed to cause a group of diseases known as prion diseases or transmissible spongiform encephalopathies. Well-known prion diseases are Creutzfeldt-Jakob disease (CJD) and kuru in humans, scrapie in sheep, bovine spongiform encephalopathy…
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Full text Article Prions

From 50 Biology Ideas You Really Need to Know
Prions cause contagious brain diseases in mammals. Unlike infectious agents such as viruses, they are not associated with nucleic acid; they are just proteins. They are not alive, so cannot be killed – they are also difficult to destroy, and there is no known cure. And yet, prions might not be all…
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Full text Article prion

From The Hutchinson Unabridged Encyclopedia with Atlas and Weather Guide
Infectious agent, a hundred times smaller than a virus. Composed of protein, and without any detectable nucleic acid (genetic material), it is strongly linked to a number of fatal degenerative brain diseases in mammals, such as bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep, and…
| 532 words
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Full text Article Prion Diseases

From Human Diseases and Conditions
AP Images/DRS. STANLEY PRUSINER FRED COHEN....
Prion diseases are fatal degenerative 3234 brain diseases that can be transmitted between species, as when meat from cattle with mad cow disease is eaten by humans; or inherited, as when a genetic mutation is passed from parent to child as in Creutzfeldt-Jakob disease (CJD). These diseases cause…
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Full text Article Prion Diseases

From The Encyclopedia of Aging
The prion diseases are a group of neurodegenerative disorders that include all transmissible spongiform encephalopathies and some related atypical forms. There are six prion diseases of animals and four of humans (Collinge & Palmer, 1997 ). The prototype is scrapie, a sporadic disease of sheep…
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Full text Article Prion Diseases

From Worldmark Global Health and Medicine Issues
SOURCE: “Creutzfeldt-Jakob Disease Deaths and...
Prion diseases, also known as transmissible spongiform encephalopathies (TSEs), are a family of rare, progressive, and fatal conditions that impair the central nervous system in both animals and humans. These diseases, as the name suggests, are caused by prions. Short for proteinaceous infectious…
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Full text Article Prion Diseases

From Encyclopedia of the Human Brain
GLOSSARY Prion diseases are rapidly progressing, invariably fatal, neurodegenerative diseases associated with dementia and neurological deficits such as ataxia, visual disturbances, or myoclonus. Histologically, nerve cell loss, spongiform change, and various forms of prion protein deposits are…
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Full text Article prion protein

From Collins Dictionary of Medicine
A protease-resistant sialoglycoprotein that is a normal constituent of the brain. Abnormal forms of the protein are now generally accepted as the causal agents in CREUTZFELDT-JAKOB DISEASE (CJD) and bovine spongiform encephalopathy (BSE). The protein was isolated by Stanley Prusiner in 1982, the…
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Full text Article Human Prion Diseases

From Brenner's Encyclopedia of Genetics
Mutations and polymorphisms associated...
Abstract Prion diseases are fatal neurodegenerative disorders that lead to spongiform degeneration of the brain and the deposition of abnormally conformed host-derived prion protein. Up to 15% of the cases are autosomal genetic and caused by multiple mutations in PRNP . Inheritance is autosomal…
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