The Role of MeCP2 in the Brain
Top Cited Papers
- 10 November 2011
- journal article
- review article
- Published by Annual Reviews in Annual Review of Cell and Developmental Biology
- Vol. 27 (1), 631-652
- https://doi.org/10.1146/annurev-cellbio-092910-154121
Abstract
Methyl-CpG binding protein 2 (MeCP2) was first identified in 1992 as a protein that binds specifically to methylated DNA. Mutations in the MECP2 gene were later found to be the cause of an autism spectrum disorder, Rett syndrome. Despite almost 20 years of research into the molecular mechanisms of MeCP2 function, many questions are yet to be answered conclusively. This review considers several key questions and attempts to evaluate the current state of evidence. For example, is MeCP2 just a methyl-CpG binding protein? Is it a multifunctional protein or primarily a transcriptional repressor? We also consider whether MeCP2, as a chromosome-binding protein, acts at specific sites within the genome or more globally, and in which cell types it is functionally important. Finally, we consider two alternative views of MeCP2 in the brain: as a regulator of brain development or as a factor that helps maintain neuronal/glial function.Keywords
This publication has 134 references indexed in Scilit:
- A Model for Neural Development and Treatment of Rett Syndrome Using Human Induced Pluripotent Stem CellsCell, 2010
- The MECP2 duplication syndromeAmerican Journal of Medical Genetics Part A, 2010
- The Role of MeCP2 in Brain Development and Neurodevelopmental DisordersCurrent Psychiatry Reports, 2010
- Synaptic circuit abnormalities of motor-frontal layer 2/3 pyramidal neurons in a mutant mouse model of Rett syndromeNeurobiology of Disease, 2010
- Dendritic spine pathologies in hippocampal pyramidal neurons from Rett syndrome brain and after expression of Rett-associated MECP2 mutationsNeurobiology of Disease, 2009
- Rett Syndrome and the Impact of MeCP2 Associated Transcriptional Mechanisms on NeurotransmissionBiological Psychiatry, 2008
- MeCP2 Binding to DNA Depends upon Hydration at Methyl-CpGMolecular Cell, 2008
- Ube3a mRNA and protein expression are not decreased in Mecp2R168X mutant miceBrain Research, 2007
- Mecp2 deficiency leads to delayed maturation and altered gene expression in hippocampal neuronsNeurobiology of Disease, 2007
- DNA methylation inhibits transcription indirectly via a methyl-CpG binding proteinCell, 1991