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Publication : Susceptibility to scrapie in mice is dependent on PrPC.

First Author  Weissmann C Year  1994
Journal  Philos Trans R Soc Lond B Biol Sci Volume  343
Issue  1306 Pages  431-3
PubMed ID  7913762 Mgi Jnum  J:28555
Mgi Id  MGI:66480 Doi  10.1098/rstb.1994.0040
Citation  Weissmann C, et al. (1994) Susceptibility to scrapie in mice is dependent on PrPC. Philos Trans R Soc Lond B Biol Sci 343(1306):431-3
abstractText  Mice devoid of functional PrP genes (Prn-p(o/o) mice) showed normal development and behaviour. When inoculated with mouse scrapie prions they remained free of scrapie symptoms for at least 18 months whereas wild-type controls all died within 6 months. No propagation of infectivity could be detected in the PrP null mice. Surprisingly, heterozygous Prn-p(o/+) mice also showed enhanced resistance to scrapie. After introduction of Syrian hamster PrP transgenes, Prn-p(o/o) mice became highly susceptible to hamster but not to mouse prions. These experiments show that PrPC, possibly at close to normal levels, is required for the usual susceptibility to scrapie and that lack of homology between incoming prions and the host's PrP genes retards disease.
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