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About Prions

A prion is a class of protein found in the brains of mammals that when misfolded is believed to cause various infectious diseases of the nervous system like bovine spongiform encephalopathy in cattle and scrapie in sheep.

All proteins fold, and can misfold, but not all misfolded proteins cause disease. Misfolded prions are different from other misfolded proteins because they can act as an infectious agent and transfer certain diseases within species.

Prion diseases are transmissible—from host to host of a single species and sometimes from one species to another—and destroy brain tissue giving it a spongy appearance. For these reasons, prion diseases are also called transmissible spongiform encephalopathies or TSEs.

Some examples of prion disease and the mammals they affect are:

• Creutzfeldt-Jakob disease (CJD; humans)
• variant Creutzfeldt-Jakob disease (vCJD; humans, acquired from cattle with BSE)
• Bovine Spongiform Encephalopathy (BSE, a.k.a. mad cow disease)
• Chronic Wasting disease (CWD; elk, deer)
• Scrapie (infectious disease of sheep and goats)
• Kuru (infectious, in humans who practiced cannibalism in Papua New Guinea)
• Gerstmann-Sträussler-Scheinker disease (inherited disease of humans)
• Fatal Familial Insomnia (inherited disease of humans)