Prion diseases and the BSE crisis

SB Prusiner - Science, 1997 - science.org
SB Prusiner
Science, 1997science.org
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 β-sheet content. It is thought that …
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 β-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.
AAAS