Goldmann W, Martin T, Foster J, Hughes S, Smith G, Hughes K, Dawson M, Hunter N
Institute for Animal Health, BBSRC and MRC Neuropathogenesis Unit, Edinburgh, UK.
J Gen Virol. 1996 Nov;77 ( Pt 11):2885-91. doi: 10.1099/0022-1317-77-11-2885.
Age at disease onset and rate of progression of transmissible spongiform encephalopathies in man, sheep and mice are modulated by the host genome, in particular by the PrP gene and its allelic forms. Analysis of the caprine PrP gene revealed several different alleles. Four PrP protein variants were found, three of which were goat specific with single amino acid changes at codons 142, 143 and 240. The fourth was identical to the most common sheep PrP protein variant (Ala136-Arg154-Gln171). The dimorphism at codon 142 (Ile --> Met) appeared to be associated with differing disease incubation periods in goats experimentally infected with isolates of bovine spongiform encephalopathy, sheep scrapie CH1641 or sheep-passaged ME7 scrapie.
人类、绵羊和小鼠传染性海绵状脑病的发病年龄和病程进展受宿主基因组调控，特别是受朊蛋白（PrP）基因及其等位基因形式的调控。对山羊PrP基因的分析揭示了几个不同的等位基因。发现了四种PrP蛋白变体，其中三种是山羊特有的，在密码子142、143和240处有单个氨基酸变化。第四种与最常见的绵羊PrP蛋白变体（Ala136-Arg154-Gln171）相同。密码子142处的二态性（异亮氨酸→甲硫氨酸）似乎与实验性感染牛海绵状脑病、绵羊瘙痒病CH1641或绵羊传代ME7瘙痒病毒株的山羊不同的疾病潜伏期有关。
