NR AKQG
AU Scott,M.R.D.; Will,R.; Ironside,J.; Nguyen,H.O.B.; Tremblay,P.; DeArmond,S.J.; Prusiner,S.B.
TI Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans
QU Proceedings of the National Academy of Sciences of the United States of America 1999 Dec 21; 96(26): 15137-42
PT journal article
AB There is growing concern that bovine spongiform encephalopathy (BSE) may have passed from cattle to humans. We report here that transgenic (Tg) mice expressing bovine (Bo) prion protein (PrP) serially propagate BSE prions and that there is no species barrier for transmission from cattle to Tg(BoPrP) mice. These same mice were also highly susceptible to a new variant of Creutzfeldt-Jakob disease (nvCJD) and natural sheep scrapie. The incubation times (approximately 250 days), neuropathology, and disease-causing PrP isoforms in Tg(BoPrP)Prnp(0/0) mice inoculated with nvCJD and BSE brain extracts were indistinguishable and differed dramatically from those seen in these mice injected with natural scrapie prions. Our findings provide the most compelling evidence to date that prions from cattle with BSE have infected humans and caused fatal neurodegeneration.
MH Animal; Brain/pathology; Cattle; Creutzfeldt-Jakob Syndrome/genetics/pathology/transmission; Encephalopathy, Bovine Spongiform/*transmission; Human; Mice; Mice, Transgenic; PrPsc Proteins/genetics/*pathogenicity; Prions/genetics/*pathogenicity; Risk; Scrapie/genetics/pathology/transmission; Species Specificity; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.; Tissue Distribution
AD Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA
SP englisch
PO USA
EA pdf-Datei und HTML-Version