NR AMIA
AU Weissmann,C.
TI Molecular genetics of transmissible spongiform encephalopathies: an introduction.
QU Journal of Toxicological Sciences 2002 May; 27(2): 69-77
PT journal article; review; review, tutorial
AB Prnp knockout mice disrupted PrPc-related genes have played an essential role to elucidate the relationship between PrPc, a normal host gene product, and PrPsc, a protease-resistant, infectious PrP; Prnp knockout mice developed by Büeler et al. (1992) were completely protected against scrapie disease when challenged with mouse prions. Further, varying expression levels in PrPc were revisited along with a varying susceptibility of mouse prions, when mouse Prnp genes were introduced into Prnp% mice. How these murine models for human prion-related disease would contribute to the presently ongoing TSE research?
ZR 67
MH Amyloid/*genetics; Animal; Creutzfeldt-Jakob Syndrome/genetics; Disease Models, Animal; Human; Mice; Mice, Knockout; Mutation; PrPc Proteins/genetics; PrPsc Proteins/genetics; Prion Diseases/*genetics; Prions/*genetics/pathogenicity; Protein Precursors/*genetics
AD MRC Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, Queen Square, London WC1N 3BG, UK
SP englisch
PO Japan