Transmissible Spongiform Encephalopathies (TSE\u27s) are neurodegenerative disorders characterized by a long generation time, spongy degeneration in the cerebral gray matter, neuronal loss and proliferation and hypertrophy of glial cells. An abnormal form of the prion protein (PrP) plays a major part in TSE pathogenesis and has been hypothesized to be the only component of the infectious agent. Some animals exposed to scrapie, the TSE affecting sheep and goats, seem to be resistant to development of the disease. Alleles encoding amino acid substitutions at codons 136 (A/V) and 171 (Q/R/H) have been associated with scrapie resistance. Other amino acid substitutions at codons 112 (M/T), 137 (M/T), 141 (L/F), 154 (R/H), and 211 (R/Q) have been reported but not associated with scrapie resistance. It may be possible to reduce the incidence of ovine scrapie by increasing the frequency of resistant genotypes (AA-136, RR-171, or QR-171). Thus, an important consideration is the frequency of these genotypes in different breeds of sheep. In this study, the genetic sequence for codons 104-175 was determined for at least ten animals of ten sheep breeds (n=207). Genotypes at codons 112, 136, 154, and 171 were determined. For codon 136, the frequency of the susceptible allele (V) was less than 0.20 in all breeds. In contrast, the frequency of the susceptible allele (Q) at codon 171 ranged from 0.27 (St. Croix) to 0.96 (Hampshire). In addition, a previously unreported substitution was found at codon 143 (H/R), with frequencies as high as 0.40
