Bovine spongiform encephalopathy (BSE), also known as "mad cow disease," is a fatal brain disease of cattle first recognized in the United Kingdom. In humans, the most common transmissible spongiform encephalopathy is Creutzfeldt-Jacob Disease (CJD).They are among the most notable central nervous system degenerative disorders caused by prions.These prion diseases which are unlike any other group of infectious diseases. Rather than being caused by a conventional micro-organism, the primary pathogenic event consists of the transformation of a normal protein (the prion protein) into an abnormal form, which can transmit disease. Prion disease is endemic in humans and sheep where it is associated with polymorphisms or mutations within the prion protein gene. Although the disease in these cases arises spontaneously, it produces an infectious prion protein. Prions are transmissible particles that are devoid of nucleic acid and seem to be composed exclusively of a modified protein (PrPSc). The normal, cellular prion protein (PrPC) is converted into PrPSc through a posttranslational process during which it acquires a high beta-sheet content. It is thought that BSE is a result of cannibalism in which faulty industrial practices produced prion-contaminated feed for cattle. There is now considerable concern that bovine prions may have been passed to humans, resulting in a new form of CJD.
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