title: The role of MeCP2 in the brain.
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.
authors: Wellcome Trust Centre for Cell Biology, University of Edinburgh, Edinburgh EH9 3JR, United Kingdom. J.Guy@ed.ac.uk
authors: Jacky
authors: J
authors: Guy
authors: Hélène
authors: H
authors: Cheval
authors: Jim
authors: J
authors: Selfridge
authors: Adrian
authors: A
authors: Bird
mesh_terms: D000818:Animals; D001921:Brain / Q000033:anatomy & histology / Q000254:growth & development / Q000378:metabolism*; D018899:CpG Islands; D019175:DNA Methylation; D006801:Humans; D051783:Methyl-CpG-Binding Protein 2 / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D009457:Neuroglia / Q000378:metabolism; D009474:Neurons / Q000378:metabolism; D011485:Protein Binding; D017434:Protein Structure, Tertiary; D012097:Repressor Proteins / Q000235:genetics / Q000378:metabolism
chemical_list: D051783:Methyl-CpG-Binding Protein 2; D012097:Repressor Proteins