Neurodegenerative Diseases Unit, ANSES-Lyon, Lyon, France.
J Neuropathol Exp Neurol. 2012 Feb;71(2):140-7. doi: 10.1097/NEN.0b013e3182439519.
A few cases of transmissible spongiform encephalopathies in sheep have been described in France in which the protease-resistant prion protein (PrP(res)) exhibited some features in Western blot of experimental bovine spongiform encephalopathy in sheep. Their molecular characteristics were indistinguishable from those produced in the CH1641 experimental scrapie isolate. Four of these CH1641-like isolates were inoculated intracerebrally into wild-type C57Bl/6 mice. In striking contrast to previous results in ovine transgenic mice, CH1641 transmission in wild-type mice was efficient. Several components of the strain signature, that is, PrP(res) profile, brain distribution, and morphology of the deposits of the disease-associated prion protein, had some similarities with "classical" scrapie and clearly differed from both bovine spongiform encephalopathy in sheep and CH1641 transmission in ovine transgenic mice. These results on CH1641-like isolates in wild-type mice may be consistent with the presence in these isolates of mixed conformers with different abilities to propagate and mediate specific disease phenotypes in different species.
在法国已经描述了几例绵羊传染性海绵状脑病病例，其中蛋白酶抗性朊病毒蛋白（PrP(res)）在绵羊实验性牛海绵状脑病的 Western blot 中表现出一些特征。它们的分子特征与 CH1641 实验性羊瘙痒病分离株产生的特征无法区分。这 4 个 CH1641 样分离株被脑内接种到野生型 C57Bl/6 小鼠中。与先前在绵羊转基因小鼠中的结果形成鲜明对比的是，CH1641 在野生型小鼠中的传播非常有效。该株的一些特征成分，即 PrP(res)谱、脑分布和疾病相关朊病毒蛋白沉积物的形态，与“经典”瘙痒病有一些相似之处，与绵羊的牛海绵状脑病和绵羊转基因小鼠中的 CH1641 传播明显不同。这些关于野生型小鼠中 CH1641 样分离株的结果可能与这些分离株中存在具有不同传播能力和在不同物种中介导特定疾病表型的混合构象体一致。
