Mice devoid of PrP are resistant to scrapie

@article{Beler1993MiceDO,
  title={Mice devoid of PrP are resistant to scrapie},
  author={Hansruedi B{\"u}eler and Adriano Aguzzi and Andreas Sailer and Romy Greiner and Charles Weissmann},
  journal={Cell},
  year={1993},
  volume={73},
  pages={1339-1347}
}
  • Hansruedi Büeler, Adriano Aguzzi, +2 authors Charles Weissmann
  • Published in Cell 1993
  • Biology, Medicine
  • 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… CONTINUE READING

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