Cell

Cell

Volume 51, Issue 4, 20 November 1987, Pages 651-662
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Article
Distinct prion proteins in short and long scrapie incubation period mice

https://doi.org/10.1016/0092-8674(87)90134-6Get rights and content

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 (ILn) and short (NZW) incubation periods, Prn-p transcription is initiated at similar multiple sites. The predicted NZW and ILn 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 ILn 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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