Methyl-CpG binding proteins in the nervous system.
Classical methyl-CpG binding proteins contain the conserved DNA binding motif methyl-cytosine binding domain (MBD), which preferentially binds to methylated CpG dinucleotides. These proteins serve as transcriptional repressors, mediating gene silencing via DNA cytosine methylation. Mutations in methyl-CpG binding protein 2 (MeCP2) have been linked to the human mental retardation disorder Rett syndrome, suggesting an important role for methyl-CpG binding proteins in brain development and function. This mini-review summarizes the recent advances in studying the diverse functions of MeCP2 as a prototype for other methyl-CpG binding proteins in the development and function of the vertebrate nervous system.
Fan
Guoping
G
Department of Human Genetics and Interdepartmental Program of Neuroscience, David Geffen School of Medicine, University of California at Los Angeles, 90095-7088, USA. gfan@mednet.ucla.edu
Hutnick
Leah
L
D000818:Animals; D002868:Chromosomal Proteins, Non-Histone / Q000235:genetics / Q000502:physiology; D018899:CpG Islands / Q000502:physiology*; D019175:DNA Methylation; D004268:DNA-Binding Proteins / Q000235:genetics / Q000502:physiology*; D006801:Humans; D051783:Methyl-CpG-Binding Protein 2; D009154:Mutation; D009420:Nervous System / Q000378:metabolism*; D012333:RNA, Messenger / Q000235:genetics; D012097:Repressor Proteins / Q000235:genetics / Q000502:physiology
D002868:Chromosomal Proteins, Non-Histone; D004268:DNA-Binding Proteins; C497392:MECP2 protein, human; D051783:Methyl-CpG-Binding Protein 2; D012333:RNA, Messenger; D012097:Repressor Proteins