We investigated the relationships between amino acid polymorphisms of the prion protein (PrP), restriction fragment length polymorphisms (RFLP) of the PrP gene and the incidence of natural scrapie in Japan. Six variant alleles of the PrP gene were found in healthy sheep. Based on the substitutions at codons 112, 136, 154 and 171, these allelic variants were designated PrP, PrP, PrP, PrP, PrP and PrP. Each RFLP haplotype (e1h1, e1h2 or e3h1) consisted of multiple alleles including PrP. Three of these variant alleles were found in scrapie-affected Suffolk sheep. PrP was associated with high disease incidence, PrP and PrP were associated with low disease incidence. We found that one scrapie-affected Suffolk was homozygous for PrP and four PrP-positive Suffolks carried PrP. Both of two scrapie-affected Corriedales and two out of three scrapie-affected cross-breeds between Suffolk and Corriedale carried PrP, suggesting that this allele associates with high incidence of scrapie in Corriedale and its cross-breeds.


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