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Updated: Jun 12, 2026

An Electrochemiluminescence-Based Assay for MeCP2 Protein Variants
Published on: May 22, 2020
High expression of MeCP2 in JC virus-infected cells of progressive multifocal leukoencephalopathy brains
Saya Shirai1, Kenta Takahashi, Shinji Kohsaka
1Laboratory of Cancer Research, Department of Pathology, Hokkaido University Graduate School of Medicine, Hokkaido, Japan.
Abstract:
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.
Insights
Methyl CpG binding protein 2 (MeCP2) is highly expressed in JC virus (JCV) infected glial cells in progressive multifocal leukoencephalopathy (PML) brains. This finding offers new insights into MeCP2 regulation in glial cells during JCV infection.
Area of Science:
- Neuroscience
- Virology
- Molecular Biology
Background:
- Mutations in the methyl CpG binding protein 2 (MeCP2) gene are a primary cause of Rett syndrome.
- JC virus (JCV) causes progressive multifocal leukoencephalopathy (PML), a demyelinating disease of the brain.
Purpose of the Study:
- To investigate the relationship between MeCP2 gene expression and JCV infection in the brain.
- To determine if JCV infection influences MeCP2 expression in glial cells.
Main Methods:
- Examination of brain tissue from four PML patients.
- Immunohistochemical labeling for JCV proteins (VP1, agnoprotein, large T antigen) to confirm infection.
- Immunohistochemistry using an antibody against MeCP2 to assess its expression.
- Double immunolabeling to co-localize JCV large T antigen and MeCP2.
Main Results:
- MeCP2 was detected in neuronal nuclei in normal and uninfected PML brain tissue.
- In PML brains, intense MeCP2 immunolabeling was observed in enlarged glial nuclei within JCV-infected cells.
- Double labeling confirmed that JCV-infected glial nuclei expressed MeCP2.
Conclusions:
- MeCP2 is highly expressed in JCV-infected glial cell nuclei within PML brain tissue.
- JCV infection may play a role in regulating MeCP2 expression in glial cells.
- These findings may offer new insights into the pathogenesis of PML and MeCP2-related disorders.
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