Distinct prion proteins in short and long scrapie incubation period mice

Analysis of linkage between scrapie incubation period and the prion protein gene in mice

“…It is possible that this linkage could be explained by a BstEII restriction site difference and accompanying amino acid sequence difference at position 189 of the PrP protein of NZW mice, compared with I/LnJ and P/J mice (Westaway et al, 1987). However, in this study and previous work there have consistently been a few mice whose incubation period phenotype and PrP-linked restriction site appeared to be separable (Carlson et al, 1986(Carlson et al, , 1988.…”

“…However, so far no differences between NZW and C57BL/6 have been detected in restriction mapping studies of PrP (Carlson et al, 1988). Furthermore, in constrast to I/LnJ and P/J mice, C57BL/6, NZW and MA/MyJ all have a BstEII site at the same position within the PrP open reading frame (Westaway et al, 1987). Thus, the linkage between PrP and incubation period in crosses of MA/MyJ and NZW mice cannot be explained on the basis of this particular genetic marker.…”

“…I/LnJ mice were known to have very long scrapie incubation periods after inoculation of Chandler scrapie agent (Carlson et al, 1986) and I/LnJ, P/J, MA/MyJ and RIIIS/J mice all shared an XbaI restriction enzyme site polymorphism located approximately 0.9 kb upstream of 0000-9065 © 1990 SGM the PrP structural gene (Westaway et al, 1987;Carlson et aI., 1988). In the present experiments each of these strains was crossed with N Z W mice, a strain known to have a short scrapie incubation time following inoculation of this isolate (Carlson et al, 1986).…”

Host Genetic Control of Incubation Periods of Creutzfeldt-Jakob Disease in Mice

“…However, differences in incubation period have been observed in mouse strains of the same Sinc s7 and/or Prn-p ~ genotype (Outram, 1976;Kingsbury et al, 1983;Westaway et al, 1987). This suggests the presence of another factor that influences the incubation period of scrapie and CJD in mice.…”

“…These genes from the prion gene complex were located on chromosome 2 (Sparkes et al, 1986). Studies of the nucleotide sequence of the Prn-p gene of inbred mouse strains revealed substitutions at codons 108 and 189 (Westaway et al, 1987). On scruple infection, NZW, C57BL/6J and many other inbred strains of mice having the same amino acids at codons 108 and 189, Prn-p ~, had short incubation periods, whereas I/Ln, IM, P/J and BDP/J mice that have one or two substitutions at codons 108 and 189, Prn-p b, had longer incubation periods.…”