Prion diseases and the BSE crisis.
Bovine spongiform encephalopathy (BSE) and human Creutzfeldt-Jakob disease (CJD) are among the most notable central nervous system degenerative disorders caused by prions. CJD may present as a sporadic, genetic, or infectious illness. 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.
Prusiner
S B
SB
Department of Neurology, University of California, San Francisco, CA 94143, USA.
D000818:Animals; D002417:Cattle; D007562:Creutzfeldt-Jakob Syndrome / Q000209:etiology / Q000235:genetics / Q000628:therapy / Q000635:transmission; D016643:Encephalopathy, Bovine Spongiform / Q000209:etiology / Q000628:therapy / Q000635:transmission; D006801:Humans; D009154:Mutation; D018621:PrPC Proteins / Q000737:chemistry / Q000235:genetics; D018620:PrPSc Proteins / Q000737:chemistry / Q000235:genetics; D017096:Prion Diseases / Q000209:etiology / Q000235:genetics / Q000628:therapy / Q000635:transmission; D011487:Protein Conformation
D018621:PrPC Proteins; D018620:PrPSc Proteins