Molecular genetics of transmissible spongiform encephalopathies: an introduction.
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?
Weissmann
Charles
C
MRC Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, Queen Square, London WC1N 3BG, UK.
D000682:Amyloid / Q000235:genetics*; D000818:Animals; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D018345:Mice, Knockout; D009154:Mutation; D018621:PrPC Proteins / Q000235:genetics; D018620:PrPSc Proteins / Q000235:genetics; D017096:Prion Diseases / Q000235:genetics*; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics* / Q000472:pathogenicity; D011498:Protein Precursors / Q000235:genetics*
D000682:Amyloid; C512656:PRNP protein, human; D018621:PrPC Proteins; D018620:PrPSc Proteins; D000072002:Prion Proteins; D011328:Prions; C512657:Prnp protein, mouse; D011498:Protein Precursors