Definition and Information

A protein fragment that becomes a pathogen. Prion illnesses affect the brain and cause extensive damage to brain tissue, causing it to become spongy in appearance. Though illnesses are not contagious in typical fashion, introduction of infectious prions into healthy brain tissue transmits the infection.

Because of these characteristics, researchers call prion illnesses transmittable spongiform encephalopathies (TSEs). Some diseases are inherited and others are acquired. The most notorious prion illness is variant Creutzfeldt-Jakob disease (vCJD), acquired through the consumption of beef from cattle that have bovine spongiform encephalopathy (BSE), commonly called mad cow disease.

Prions are highly resistant to disinfectants and sterilization procedures. Many hospitals now use disposable instruments for brain surgery to reduce the risk for transmitting a prion disease through an operation.

See also CREUTZFELDT-JAKOB DISEASE (CJD); FOOD SAFETY.

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