Science, Vol.278, No.5336, 245-251, 1997
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.
Keywords:CREUTZFELDT-JAKOB-DISEASE;BOVINE SPONGIFORM ENCEPHALOPATHY;GERSTMANN-STRAUSSLER SYNDROME;TRANSMISSIBLE MINK ENCEPHALOPATHY;SCRAPIE-ASSOCIATED PROTEIN;MICE EXPRESSING HUMAN;TRANSGENIC MICE;STRAIN VARIATION;PRP GENE;INCUBATION-TIME