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

Moore, R C; Hope, J; McBride, P A; McConnell, I; Selfridge, J; Melton, D W; Manson, J C

Moore, R C; Hope, J; McBride, P A; McConnell, I; Selfridge, J; Melton, D W; Manson, J C.

Afiliación

Moore RC; Institute of Cell and Molecular Biology, University of Edinburgh, UK.

Nat Genet ; 18(2): 118-25, 1998 Feb.

Article en En | MEDLINE | ID: mdl-9462739

RESUMEN

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.

Asunto(s)

Priones/genética; Alelos; Animales; Encéfalo/metabolismo; Encéfalo/patología; Codón; Variación Genética; Ratones; Ratones Mutantes; Ratones Transgénicos; Datos de Secuencia Molecular; Mutación Puntual; Priones/biosíntesis; Priones/química; Scrapie/genética; Scrapie/patología; Especificidad de la Especie

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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Priones Límite: Animals Idioma: En Revista: Nat Genet Asunto de la revista: GENETICA MEDICA Año: 1998 Tipo del documento: Article Pais de publicación: Estados Unidos

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