MeCP2 in central nervous system glial cells: current updates

• Published in: Acta neurobiologiae experimentalis Vol. 78; no. 1; pp. 30 - 40
• Main Authors: Sharma, Kedarlal, Singh, Juhi, Frost, Emma E., Pillai, Prakash P.
• Format: Journal Article
• Language: English
• Published: Poland Polish Academy of Sciences 01.01.2018
• Subjects: Animals; Astrocytes; Central nervous system; Central Nervous System - cytology; Central Nervous System - pathology; CpG islands; Deoxyribonucleic acid; DNA; Glial cells; Humans; MeCP2 protein; Methyl-CpG binding protein; Methyl-CpG-Binding Protein 2 - genetics; Methyl-CpG-Binding Protein 2 - metabolism; Microglia; Mutation; Neurodevelopmental disorders; Neuroglia - metabolism; Neuronal-glial interactions; Oligodendrocytes; Pathogenesis; Proteins; Rett syndrome; Rett Syndrome - genetics; Rett Syndrome - pathology; Upgrading
• ISSN: 0065-1400; 1689-0035; 1689-0035
• DOI: 10.21307/ane-2018-007

avMethyl‑CpG binding protein 2 (MeCP2) is an epigenetic regulator, which preferentially binds to methylated CpG dinucleotides in DNA. MeCP2 mutations have been linked to Rett syndrome, a neurodevelopmental disorder characterized by severe intellectual disability in females. Earlier studies indicated that loss of MeCP2 function in neuronal cells was the sole cause of Rett syndrome. Subsequent studies have linked MeCP2 expression in CNS glial cells to Rett syndrome pathogenesis. In this review, we have discussed the role of MeCP2 in glial subtypes, astrocytes, oligodendrocytes and microglia, and how loss of MeCP2 function in these cells has a profound influence on both glial and neuronal function.