Mice devoid of PrP are resistant to scrapie

@article{Beler1993MiceDO,
  title={Mice devoid of PrP are resistant to scrapie},
  author={H. B{\"u}eler and A. Aguzzi and A. Sailer and R. Greiner and C. Weissmann},
  journal={Cell},
  year={1993},
  volume={73},
  pages={1339-1347}
}
S.B. Prusiner proposed that the infectious agent of scraple, the prion, is PrPSc, a modified form of the normal host protein PrPC. Prn-p0/0 mice devoid of PrPC showed normal development and behavior. When inoculated with mouse scrapie prions, they remained free of scrapie symptoms for at least 13 months while wild-type controls all died within 6 months. Surprisingly, heterozygous Prn-p0/+ mice also showed enhanced resistance to scrapie. After introduction of Syrian hamster PrP transgenes, Prn… Expand
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Paper Mentions

Observational Clinical Trial
The study hypothesis is that that the deleterious effect of prions on the brain may be mediated (at least partially) by activation of serine proteases involved in the coagulation… Expand
ConditionsCreutzfeldt-Jakob Syndrome
InterventionProcedure
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