Laboratory of Cancer Research, Department of Pathology, Hokkaido University Graduate School of Medicine, Hokkaido, Japan.
Neuropathology. 2011 Feb;31(1):38-41. doi: 10.1111/j.1440-1789.2010.01122.x.
Mutations of the methyl CpG binding protein 2 (MeCP2) gene are a major cause of Rett syndrome. To investigate whether the expression of this gene was related to JC virus (JCV) infection, we examined brains of four progressive multifocal leukoencephalopathy (PML) patients. JCV infection was confirmed by immunohistochemical labeling with antibodies against JCV VP1, agnoprotein and large T antigen. MeCP2 expression was examined by immunohistochemistry using a specific polyclonal antibody against MeCP2. In normal brains and uninfected cortices of PML brains, MeCP2 expression was observed in the nuclei of neurons, but not observed in glial and endothelial cell nuclei. However, in PML brains intense immunolabeling was observed in abnormally enlarged glial nuclei of JCV-infected cells. Double immunolabeling using antibodies against large T antigen (visualized as blue) and MeCP2 (visualised as red) revealed dark red JCV-infected nuclei, which confirmed that the JCV infected nuclei expressed MeCP2. We conclude that MeCP2 is highly expressed in the JCV-infected nuclei of PML brain and these results may provide a new insight into the mechanism which regulates the MeCP2 expression in glial cells by the infection of JCV.
甲基 CpG 结合蛋白 2(MeCP2) 基因突变是 Rett 综合征的主要病因。为了研究该基因的表达是否与 JC 病毒(JCV)感染有关，我们检查了 4 例进行性多灶性白质脑病(PML)患者的大脑。通过用针对 JCV VP1、agnoprotein 和大 T 抗原的抗体进行免疫组织化学标记来确认 JCV 感染。使用针对 MeCP2 的特异性多克隆抗体通过免疫组织化学检查 MeCP2 的表达。在正常大脑和 PML 大脑未感染的皮质中，MeCP2 在神经元核中表达，但在神经胶质和内皮细胞核中未观察到。然而，在 PML 大脑中，在 JCV 感染细胞中异常增大的神经胶质核中观察到强烈的免疫标记。使用针对大 T 抗原(显示为蓝色)和 MeCP2(显示为红色)的双重免疫标记揭示了深红色的 JCV 感染核，这证实了 JCV 感染核表达了 MeCP2。我们得出结论，MeCP2 在 PML 脑的 JCV 感染核中高度表达，这些结果可能为 JCV 感染调节神经胶质细胞中 MeCP2 表达的机制提供新的见解。
