Distinct prion proteins in short and long scrapie incubation period mice
D Westaway, PA Goodman, CA Mirenda, MP McKinley… - Cell, 1987 - cell.com
Cell, 1987•cell.com
The Pm-i gene, controlling scrapie incubation period, is linked to or congruent with the
murine prion protein (PrP) gene, Pm-p. In prototypic mouse strains with long (IILn) and short
(NZW) incubation periods, Pm-p transcription is initiated at similar multiple sites. The
predicted NZW and IlLn 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 IlLn and two …
murine prion protein (PrP) gene, Pm-p. In prototypic mouse strains with long (IILn) and short
(NZW) incubation periods, Pm-p transcription is initiated at similar multiple sites. The
predicted NZW and IlLn 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 IlLn and two …
Summary
The Pm-i gene, controlling scrapie incubation period, is linked to or congruent with the murine prion protein (PrP) gene, Pm-p. In prototypic mouse strains with long (IILn) and short (NZW) incubation periods, Pm-p transcription is initiated at similar multiple sites. The predicted NZW and IlLn 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 IlLn 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 Pm-p and Pm-i.
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