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Molecular genetics of transmissible spongiform encephalopathies: an introduction
1MRC Prion Unit, Department of Neurodegenerative Disease, Institute of Neurology, Queen Square, London WC1N 3BG, UK.
The Journal of Toxicological Sciences
|June 13, 2002
Abstract:
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?