MeCP2: multifaceted roles in gene regulation and neural development.

作者: Tian-Lin Cheng , Zilong Qiu

DOI: 10.1007/S12264-014-1452-6

关键词: Neural developmentDGCR8DroshaMECP2Regulation of gene expressionRett syndromePost-transcriptional regulationGeneticsBiologyNeuroscienceSUMO protein

摘要: Methyl-CpG-binding protein 2 (MeCP2) is a classic methylated-DNA-binding protein, dysfunctions of which lead to various neurodevelopmental disorders such as Rett syndrome and autism spectrum disorder. Initially recognized transcriptional repressor, MeCP2 has been studied extensively its functions have expanded dramatically in the past two decades. Recently, it was found be involved gene regulation at post-transcriptional level. represses nuclear microRNA processing by interacting directly with Drosha/DGCR8 complex. In addition multifaceted functions, remarkably modulated posttranslational modifications phosphorylation, SUMOylation, acetylation, providing more regulatory dimensions functions. The role central nervous system extensively, from neurons glia. Future investigations combining molecular, cellular, physiological methods are necessary for defining roles brain developing efficient treatments MeCP2-related disorders.

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