Faculty Research 1980 - 1989

Title

Distinct prion proteins in short and long scrapie incubation period mice.

Document Type

Article

Publication Date

1987

Keywords

Animal, Base-Sequence, Codon, Comparative-Study, Disease-Susceptibility, Mice, Mice-Inbred-Strains, Molecular-Sequence-Data, Polymorphism-(Genetics), Prions: ge, Scrapie-Agent: ge, Scrapie: et, pp, pa, SUPPORT-NON-U-S-GOVT, SUPPORT-U-S-GOVT-P-H-S, Time-Factors, Viral-Proteins: ge

JAX Source

Cell 1987 Nov 20; 51(4):651-62.

Grant

AG02132, NS14069, NS22786

Abstract

The Prn-i gene, controlling scrapie incubation period, is linked to or congruent with the murine prion protein (PrP) gene, Prn-p. In prototypic mouse strains with long (l/Ln) and short (NZW) incubation periods, Prn-p transcription is initiated at similar multiple sites. The predicted NZW and l/Ln PrP proteins differ at codons 108 and 189. Codon 189, highly conserved in mammals, lies within a polymorphic BstEll site that is retained in 17 mouse strains known to have short or intermediate incubation times, but is absent in l/Ln and two other inbred mice with long incubation times. Codon 108 in mice with short or intermediate incubation times encodes Leu; in mice with long incubation times it encodes Phe. The correlation of PrP sequence with length of scrapie incubation period suggests, but does not formally prove, congruency between Prn-p and Prn-i.

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