Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies.

@article{Prusiner1993AblationOT,
  title={Ablation of the prion protein (PrP) gene in mice prevents scrapie and facilitates production of anti-PrP antibodies.},
  author={Stanley B Prusiner and Darlene F Groth and Ana V. Serban and Rolf K{\"o}hler and Don Foster and Marilyn Torchia and Doris Burton and Sh. L. Yang and Stephen J. DeArmond},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={1993},
  volume={90 22},
  pages={10608-12}
}
Mice, homozygous for prion protein (PrP) gene ablation (Prn-p0/0), develop normally and remain well > 500 days after inoculation with murine scrapie prions. In contrast, wild-type mice developed scrapie < 165 days after inoculation and most Prn-p0/+ mice, heterozygous for disruption of the PrP gene, exhibited signs of central nervous system dysfunction between 400 and 465 days after inoculation. In situ immunoblots showed widespread deposition of scrapie PrP (PrPSc) in the brains of both wild… CONTINUE READING

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