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| MICE DEVOID OF PRP ARE RESISTANT TO SCRAPIE |
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| Author(s): BUELER H, AGUZZI A, SAILER A, GREINER RA, AUTENRIED P, AGUET M, WEISSMANN C |
| Source: CELL Volume: 73 Issue: 7 Pages: 1339-1347 Published: JUL 2 1993 |
| Times Cited: 1,103 References: 40 |
| Abstract: S. B. Prusiner proposed that the infectious agent of scrapie, the prion, is PrP(Sc), a modified form of the normal host protein PrP(C). Prn-p0/0 mice devoid of PrP(C) 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 PrP(C), 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. |
| Document Type: Article |
| Language: English |
| Reprint Address: BUELER, H (reprint author), UNIV ZURICH, INST MOLEK BIOL 1, CH-8093 ZURICH, SWITZERLAND |
Addresses:
1. UNIV HOSP ZURICH, INST NEUROPATHOL, CH-8091 ZURICH, SWITZERLAND
2. UNIV HOSP ZURICH, BIOL ZENT LAB, CH-8091 ZURICH, SWITZERLAND |
| Publisher: CELL PRESS, 1050 MASSACHUSETTES AVE, CIRCULATION DEPT, CAMBRIDGE, MA 02138 |
| Subject Category: Biochemistry & Molecular Biology; Cell Biology |
| IDS Number: LL209 |
| ISSN: 0092-8674 |
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