NR AJQL
AU Prusiner,S.B.
TI Prion diseases and the BSE crisis
QU Science 1997 Oct 10; 278(5336): 245-51
IA http://www.sciencemag.org/cgi/content/full/278/5336/245
PT journal article; review; review, tutorial
AB 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.
ZR 110
MH Animal; Cattle; *Creutzfeldt-Jakob Syndrome/etiology/genetics/therapy/transmission; *Encephalopathy, Bovine Spongiform/etiology/therapy/transmission; Human; Mutation; *PrPc Proteins/chemistry/genetics; *PrPsc Proteins/chemistry/genetics; *Prion Diseases/etiology/genetics/therapy/transmission; Protein Conformation; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.
AD Department of Neurology, University of California, San Francisco, CA 94143, USA
SP englisch
PO USA
EA pdf-Datei und HTML-Version
OR Prion-Krankheiten 6