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| Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent |
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| Author(s): Moore RC, Hope J, McBride PA, McConnell I, Selfridge J, Melton DW, Manson JC |
| Source: NATURE GENETICS Volume: 18 Issue: 2 Pages: 118-125 Published: FEB 1998 |
| Times Cited: 99 References: 54 |
| Abstract: Classical genetic analysis has identified Sinc/Prni as the major gene controlling mouse scrapie incubation time. Sinc/Pmi is linked to Prnp, the gene encoding the prion protein (PrP). Prnp alleles express distinct PrP protein variants, PrP A and PrP B, which arise from codon 108L/F and 189 T/V dimorphisms. Prnp genotype segregates with incubation time length which suggests, but does not prove, that incubation time is controlled by PrP dimorphisms, and that the Sinc/Prni and Prnp loci are congruent. We have used gene targetting to construct mice in which the endogenous Prnp allele has been modified to express PrP 8 instead of PrP A. Challenge with a mouse-adapted BSE strain results in dramatically shortened incubation times and demonstrates that PrP dimorphisms at codon 108 and/or 189 control incubation time, and that Sinc/Prni and Prnp are congruent. |
| Document Type: Article |
| Language: English |
| Reprint Address: Manson, JC (reprint author), Inst Anim Hlth, Neuropathogenesis Unit, Ogston Bldg,W Mains Rd, Edinburgh EH9 3JF, Midlothian Scotland |
Addresses:
1. Inst Anim Hlth, Neuropathogenesis Unit, Edinburgh EH9 3JF, Midlothian Scotland
2. Univ Edinburgh, Inst Cell & Mol Biol, Edinburgh EH9 3JR, Midlothian Scotland
3. Inst Anim Hlth, Compton Lab, Newbury RG20 7NN, Berks England |
| Publisher: NATURE AMERICA INC, 345 PARK AVE SOUTH, NEW YORK, NY 10010-1707 USA |
| Subject Category: Genetics & Heredity |
| IDS Number: YV010 |
| ISSN: 1061-4036 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |