Prion Disease Research Center, National Institute of Animal Health, Tsukuba, Ibaraki, Japan.
Prion. 2011 Apr-Jun;5(2):103-8. doi: 10.4161/pri.5.2.15847. Epub 2011 Apr 1.
Atypical forms of bovine spongiform encephalopathy (BSE) may be caused by different prions from classical BSE (C-BSE). In this study, we examined the susceptibility of mice overexpressing mouse and hamster chimeric prion protein (PrP) to L-type atypical BSE (L-BSE). None of the transgenic mice showed susceptibility to L-BSE, except mice overexpressing hamster PrP. We also examined the transmission properties of L-BSE in hamsters. The incubation period of hamsters intracerebrally inoculated with L-BSE was 576.8 days, and that of the subsequent passage was decreased to 208 days. Although the lesion and glycoform profiles and relative proteinase K resistant core fragment of the abnormal isoform of PrP (PrPcore) of L-BSE were similar to that of C-BSE, the deposition of the abnormal isoform of PrP (PrPSc) and the molecular weight of PrPcore of L-BSE was different from than that of C-BSE. In hamster models, some prion strain characteristics of L-BSE were indistinguishable from those of C-BSE.
非典型牛海绵状脑病（BSE）的形式可能是由与经典 BSE（C-BSE）不同的朊病毒引起的。在这项研究中，我们研究了过表达鼠/仓鼠嵌合朊病毒蛋白（PrP）的小鼠对 L 型非典型 BSE（L-BSE）的易感性。除了过表达仓鼠 PrP 的小鼠外，没有一种转基因小鼠对 L-BSE 表现出易感性。我们还研究了 L-BSE 在仓鼠中的传播特性。用 L-BSE 脑内接种的仓鼠的潜伏期为 576.8 天，随后的传代潜伏期缩短至 208 天。尽管 L-BSE 的病变和糖型谱以及异常 PrP （PrPcore）的相对蛋白水解酶抗性核心片段和 C-BSE 相似，但 L-BSE 的异常 PrP （PrPSc）沉积和 PrPcore 的分子量与 C-BSE 不同。在仓鼠模型中，L-BSE 的一些朊病毒株特性与 C-BSE 无法区分。
