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. 1993 Jul 2;73(7):1339-47.
doi: 10.1016/0092-8674(93)90360-3.

Mice devoid of PrP are resistant to scrapie

H Büeler  1 , A Aguzzi, A Sailer, R A Greiner, P Autenried, M Aguet, C Weissmann
Affiliations

Mice devoid of PrP are resistant to scrapie

H Büeler et al. Cell. .

Abstract

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-p0/0 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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Comment in

  • A mouse to remember.
    Weissmann C, Büeler H. Weissmann C, et al. Cell. 2004 Jan 23;116(2 Suppl):S111-3, 2 p following S113. doi: 10.1016/s0092-8674(04)00032-7. Cell. 2004. PMID: 15055597 No abstract available.

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