Mice with gene targetted prion protein alterations show that PRNP, Sinc and Prni are congruent

Moore, Richard C., Hope, James, McBride, Patricia A., McConnell, Irene, Selfridge, James, Melton, David W. and Manson, Jean C. (1998) Mice with gene targetted prion protein alterations show that PRNP, Sinc and Prni are congruent. Nature Genetics, 18 (2). pp. 118-125. ISSN 1061-4036

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Abstract

Classical genetic analysis has identified Sinc/Prni as the major gene controlling mouse scrapie incubation time. Sinc/Prni 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 B 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.

Item Type:	Article
Uncontrolled Keywords:	genetics ,/dk/atira/pure/subjectarea/asjc/1300/1311
Faculty \ School:	Faculty of Science > School of Biological Sciences
Related URLs:	http://www.scopus.com/inward/record.url?...
Depositing User:	LivePure Connector
Date Deposited:	08 Dec 2025 16:30
Last Modified:	14 Dec 2025 07:31
URI:	https://ueaeprints.uea.ac.uk/id/eprint/101315
DOI:	10.1038/ng0298-118

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