prion

prion prēˈŏn [key], abnormal form of a protein found in mammals, now generally believed to cause a group of diseases known as prion diseases or transmissible spongiform encephalopathies, which are rare progressive degenerative neurological disorders. Well-known prion diseases are Creutzfeldt-Jakob disease (CJD) and kuru in humans, scrapie in sheep, bovine spongiform encephalopathy (BSE), also called “mad cow disease,” in cattle, and chronic wasting disease in deer, elk (wapiti), moose, and caribou. There is no effective treatment for any prion disease.

Sometimes taking more than 30 years to display symptoms, the diseases slowly attack brain tissue, often leaving spongelike holes. They are characterized by accumulations of prions, abnormal forms of a protein found on many cell surfaces and called prion protein. Unlike viruses or bacteria, prions contain no genetic material and have no known ability to reproduce themselves. Prions differ in shape from normal prion proteins due to misfolding, and are not susceptible to enzymes that normally break down proteins. In the brain, prions appear to increase their number by directly causing normal prion proteins to fold abnormally. In humans, the damage to the brain causes changes in memory, personality, and behavior, typically quickly progressing once symptoms become evident to dementia and problems with movement such as difficulty in coordination.

Prion diseases have both infectious and hereditary components. The gene that codes for prion proteins can mutate and be passed on to the next generation. Most of the diseases also can be acquired directly by infection with prions, but unlike other infectious agents, prions provoke no immune response. Most prion diseases, however, are not highly transmissable; chronic wasting disease is the exception because infected deer that have not developed the disease shed prions from lymph tissue in their intestines, contaminating the soil and plants on which other deer graze with the prions in their feces.

An epidemic of BSE in Great Britain that was diagnosed in 1986 and infected some 178,000 cows appears to have been caused by a protein feed supplement that contained rendered remains of scrapie-infected sheep brains. In 1996 a suspicion that BSE had been transmitted to humans who died of a variant of CJD in Britain caused a scientific and economic furor as the European Union imposed a ban (1996) on the export of British beef, which was partially lifted in 1999 and fully lifted in 2006. The U.S. Dept. of Agriculture banned the import of cattle and many cattle byproducts from most European nations because of BSE. Instances of BSE in cattle have also occurred in many other European countries, Canada, the United States, and Japan, but the vast majority of cases occurred in Britain in the 1980s. There is now compelling evidence that BSE is the same disease as variant CJD (vCJD), which has killed less than 200 people, but it is not yet known exactly how the disease is passed from animals to humans.

The idea of disease-causing protein particles was first put forward in 1981 by Stanley B. Prusiner, the neurologist who coined the term prion (from proteinaceous infectious particle). The prion theory was controversial from the beginning, but it is now generally accepted that prions can cause abnormal folding in normal brain prion protein, and that abnormal proteins clump and cause brain damage. Many aspects of prion diseases, however, are still poorly understood.

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