First Author | Moore RC | Year | 1998 |
Journal | Nat Genet | Volume | 18 |
Issue | 2 | Pages | 118-25 |
PubMed ID | 9462739 | Mgi Jnum | J:45908 |
Mgi Id | MGI:1196690 | Doi | 10.1038/ng0298-118 |
Citation | Moore RC, et al. (1998) Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent [see comments]. Nat Genet 18(2):118-25 |
abstractText | 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. |