title: MeCP2 in Rett syndrome: transcriptional repressor or chromatin architectural protein?
abstract: Rett syndrome is a progressive neurological disorder caused by mutations in the methyl-DNA binding protein MeCP2. The longstanding model depicting MeCP2 as a transcriptional repressor predicts that the Rett syndrome phenotype probably results from misregulation of MeCP2 target genes. Somewhat unexpectedly, the identification of such targets has proven challenging. The recent identification of two MeCP2 targets, BDNF and DLX5, are suggestive of two very different roles for this protein--one as a classical repressor protein, and the other as a mediator of a complex, specialized chromatin structure.
authors.affiliation: Laboratory of Molecular Carcinogenesis, National Institute of Environmental Health Sciences, Research Triangle Park, NC 27709, USA.
authors.forename: Lisa Helbling
authors.initials: LH
authors.lastname: Chadwick
authors.forename: Paul A
authors.initials: PA
authors.lastname: Wade
mesh_terms: D019208:Brain-Derived Neurotrophic Factor / Q000235:genetics; D002843:Chromatin / Q000235:genetics*; D005786:Gene Expression Regulation / Q000235:genetics*; D018398:Homeodomain Proteins / Q000235:genetics; D006801:Humans; D051783:Methyl-CpG-Binding Protein 2 / Q000235:genetics*; D008957:Models, Genetic; D009154:Mutation / Q000235:genetics; D015518:Rett Syndrome / Q000235:genetics*; D014157:Transcription Factors / Q000235:genetics
chemical_list: D019208:Brain-Derived Neurotrophic Factor; D002843:Chromatin; C100596:DLX5 protein, human; D018398:Homeodomain Proteins; C497392:MECP2 protein, human; D051783:Methyl-CpG-Binding Protein 2; D014157:Transcription Factors