Prion

Article By:

Priola, Suzette A. Rocky Mountain Laboratories, National Institute of Allergy and Infectious Diseases, Laboratory of Persistent Viral Diseases, Hamilton, Montana.

Last reviewed:June 2021

DOI:https://doi.org/10.1036/1097-8542.545350

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• Animal prions, published October 2011:Download PDF Get Adobe Acrobat Reader

Any of a group of infectious proteins that cause fatal neurodegenerative diseases in humans and other animals. Prions, whose name is derived from the term proteinaceous infectious particles (Fig. 1), are proteins that, when abnormal, prompt normal brain proteins to misfold. Abnormal prions are often pathogenic and can trigger misfolding and aggregation of normal (cellular) prion proteins, which are located predominantly in the brain. It is thought that abnormal prion folding and aggregation cause vacuolation of the brain's gray matter and widespread loss of nerve cells. Prion diseases, or transmissible spongiform encephalopathies (TSEs), are rare and fatal brain disorders occurring in animals, including humans. Notable neurodegenerative diseases caused by prions are scrapie and bovine spongiform encephalopathy (mad cow disease) in animals and Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease in humans. See also: Brain; Degenerative neural diseases; Infectious disease; Nervous system disorders; Prion disease; Protein; Protein folding; Scrapie

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