MeCP2 and other methyl-CpG binding proteins.

DNA methylation is an epigenetic modification that is implicated in transcriptional silencing. Recently, it has become increasingly clear that both correct levels and proper interpretation of methylation are important factors for normal development and function of the human organism. One example is the neurological disorder Rett syndrome (RTT), which affects approximately one in 10,000 girls. RTT is caused by mutations in MeCP2, a protein that was identified by its ability to bind specifically to CpG-methylated DNA. Furthermore, MeCP2 represses transcription in a methylation-dependent manner, and it is the founding member of the family of methyl-CpG binding domain (MBD) proteins.

[1]  A. Bird,et al.  The p120 catenin partner Kaiso is a DNA methylation-dependent transcriptional repressor. , 2001, Genes & development.

[2]  E. Hoffman,et al.  MeCP2 mutations in children with and without the phenotype of Rett syndrome , 2001, Neurology.

[3]  Keith D Robertson,et al.  DNA methylation, methyltransferases, and cancer , 2001, Oncogene.

[4]  M. Nakao,et al.  Solution Structure of the Methyl-CpG Binding Domain of Human MBD1 in Complex with Methylated DNA , 2001, Cell.

[5]  Rudolf Jaenisch,et al.  Synergism of Xist Rna, DNA Methylation, and Histone Hypoacetylation in Maintaining X Chromosome Inactivation , 2001, The Journal of cell biology.

[6]  K. Zhao,et al.  MECP2 truncating mutations cause histone H4 hyperacetylation in Rett syndrome. , 2001, Human molecular genetics.

[7]  A. Wolffe,et al.  Selective association of the methyl-CpG binding protein MBD2 with the silent p14/p16 locus in human neoplasia , 2001, Proceedings of the National Academy of Sciences of the United States of America.

[8]  A. Bird,et al.  Closely related proteins MBD2 and MBD3 play distinctive but interacting roles in mouse development. , 2001, Genes & development.

[9]  R. Jaenisch,et al.  Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice , 2001, Nature Genetics.

[10]  A. Bird,et al.  DNA Recognition by the Methyl-CpG Binding Domain of MeCP2* , 2001, The Journal of Biological Chemistry.

[11]  Matthew Tudor,et al.  Loss of genomic methylation causes p53-dependent apoptosis and epigenetic deregulation , 2001, Nature Genetics.

[12]  A. Bird,et al.  A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome , 2001, Nature Genetics.

[13]  M. Grunstein,et al.  25 years after the nucleosome model: chromatin modifications. , 2000, Trends in biochemical sciences.

[14]  O. Ammerpohl,et al.  The Minimal Repression Domain of MBD2b Overlaps with the Methyl-CpG-binding Domain and Binds Directly to Sin3A* , 2000, The Journal of Biological Chemistry.

[15]  A. Wolffe,et al.  Functional consequences of Rett syndrome mutations on human MeCP2. , 2000, Nucleic acids research.

[16]  A. Bellacosa,et al.  Biphasic Kinetics of the Human DNA Repair Protein MED1 (MBD4), a Mismatch-specific DNA N-Glycosylase* , 2000, The Journal of Biological Chemistry.

[17]  T. Bestor,et al.  The DNA methyltransferases of mammals. , 2000, Human molecular genetics.

[18]  J. Ahringer.  NuRD and SIN3 histone deacetylase complexes in development. , 2000, Trends in genetics : TIG.

[19]  F. Ishikawa,et al.  MBD2‐MBD3 complex binds to hemi‐methylated DNA and forms a complex containing DNMT1 at the replication foci in late S phase , 2000, Genes to cells : devoted to molecular & cellular mechanisms.

[20]  M. Nakao,et al.  Tsutomu by Methyl-CpG Binding Protein MBD 1 Mechanism of Transcriptional Regulation , 2000 .

[21]  W. Strätling,et al.  Histone deacetylase-independent transcriptional repression by methyl-CpG-binding protein 2. , 2000, Nucleic acids research.

[22]  A. Wolffe,et al.  MeCP2 driven transcriptional repression in vitro: selectivity for methylated DNA, action at a distance and contacts with the basal transcription machinery. , 2000, Nucleic acids research.

[23]  R. Meehan,et al.  Transient depletion of xDnmt1 leads to premature gene activation in Xenopus embryos. , 2000, Genes & development.

[24]  A. Bird,et al.  Active Repression of Methylated Genes by the Chromosomal Protein MBD1 , 2000, Molecular and Cellular Biology.

[25]  A. Bird,et al.  Mammalian methyltransferases and methyl-CpG-binding domains: proteins involved in DNA methylation. , 2000, Current topics in microbiology and immunology.

[26]  A. Bird,et al.  Somatic frameshift mutations in the MBD4 gene of sporadic colon cancers with mismatch repair deficiency , 1999, Oncogene.

[27]  C. Wijmenga,et al.  The DNMT3B DNA methyltransferase gene is mutated in the ICF immunodeficiency syndrome. , 1999, Proceedings of the National Academy of Sciences of the United States of America.

[28]  M. Nakao,et al.  Solution structure of the methyl‐CpG‐binding domain of the methylation‐dependent transcriptional repressor MBD1 , 1999, The EMBO journal.

[29]  A. Bird,et al.  Methylation-Induced Repression— Belts, Braces, and Chromatin , 1999, Cell.

[30]  L. Aaltonen,et al.  The DNA repair gene MBD4 (MED1) is mutated in human carcinomas with microsatellite instability , 1999, Nature Genetics.

[31]  D. Haber,et al.  DNA Methyltransferases Dnmt3a and Dnmt3b Are Essential for De Novo Methylation and Mammalian Development , 1999, Cell.

[32]  H. Zoghbi,et al.  Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2 , 1999, Nature Genetics.

[33]  A. Bird,et al.  The thymine glycosylase MBD4 can bind to the product of deamination at methylated CpG sites , 1999, Nature.

[34]  M. Robinson,et al.  Telomere shortening and apoptosis in telomerase-inhibited human tumor cells. , 1999, Genes & development.

[35]  P N Barlow,et al.  The solution structure of the domain from MeCP2 that binds to methylated DNA. , 1999, Journal of molecular biology.

[36]  M. Nakao,et al.  Methylation-Mediated Transcriptional Silencing in Euchromatin by Methyl-CpG Binding Protein MBD1 Isoforms , 1999, Molecular and Cellular Biology.

[37]  E. Ballestar,et al.  Mi-2 complex couples DNA methylation to chromatin remodelling and histone deacetylation , 1999, Nature Genetics.

[38]  S. Cross,et al.  Genomic structure and chromosomal mapping of the murine and human Mbd1, Mbd2, Mbd3, and Mbd4 genes , 1999, Mammalian Genome.

[39]  Paul Tempst,et al.  MBD2 is a transcriptional repressor belonging to the MeCP1 histone deacetylase complex , 1999, Nature Genetics.

[40]  R. Kornberg,et al.  Twenty-Five Years of the Nucleosome, Fundamental Particle of the Eukaryote Chromosome , 1999, Cell.

[41]  A. Bird,et al.  Analysis of the NuRD subunits reveals a histone deacetylase core complex and a connection with DNA methylation. , 1999, Genes & development.

[42]  D. Ayer,et al.  Histone deacetylases: transcriptional repression with SINers and NuRDs. , 1999, Trends in cell biology.

[43]  E. Golemis,et al.  MED1, a novel human methyl-CpG-binding endonuclease, interacts with DNA mismatch repair protein MLH1. , 1999, Proceedings of the National Academy of Sciences of the United States of America.

[44]  M. Szyf,et al.  A mammalian protein with specific demethylase activity for mCpG DNA , 1999, Nature.

[45]  A. Bird,et al.  Identification and Characterization of a Family of Mammalian Methyl-CpG Binding Proteins , 1998, Molecular and Cellular Biology.

[46]  J. Strouboulis,et al.  Methylated DNA and MeCP2 recruit histone deacetylase to repress transcription , 1998, Nature Genetics.

[47]  Colin A. Johnson,et al.  Transcriptional repression by the methyl-CpG-binding protein MeCP2 involves a histone deacetylase complex , 1998, Nature.

[48]  N. C. Schanen,et al.  Neonatal Encephalopathy in Two Boys in Families With Recurrent Rett Syndrome , 1998, Journal of child neurology.

[49]  R. Jaenisch,et al.  Baculovirus-mediated expression and characterization of the full-length murine DNA methyltransferase. , 1997, Nucleic acids research.

[50]  S. Cross,et al.  A component of the transcriptional represser MeCP1 shares a motif with DNA methyltransferase and HRX proteins , 1997, Nature Genetics.

[51]  Stefan U Kass,et al.  DNA methylation directs a time-dependent repression of transcription initiation , 1997, Current Biology.

[52]  A. Bird,et al.  MeCP2 Is a Transcriptional Repressor with Abundant Binding Sites in Genomic Chromatin , 1997, Cell.

[53]  A. Bird,et al.  The methyl-CpG binding protein MeCP2 is essential for embryonic development in the mouse , 1996, Nature Genetics.

[54]  A. Bird,et al.  DNA methylation specifies chromosomal localization of MeCP2 , 1996, Molecular and cellular biology.

[55]  S. Cross,et al.  CpG islands and genes. , 1995, Current opinion in genetics & development.

[56]  S. Cross,et al.  Genetic and physical mapping of a gene encoding a methyl CpG binding protein, Mecp2, to the mouse X chromosome. , 1994, Genomics.

[57]  A. Bird,et al.  Dissection of the methyl-CpG binding domain from the chromosomal protein MeCP2. , 1993, Nucleic acids research.

[58]  A. Bird,et al.  Characterization of MeCP2, a vertebrate DNA binding protein with affinity for methylated DNA. , 1992, Nucleic acids research.

[59]  Rudolf Jaenisch,et al.  Targeted mutation of the DNA methyltransferase gene results in embryonic lethality , 1992, Cell.

[60]  A. Bird,et al.  Purification, sequence, and cellular localization of a novel chromosomal protein that binds to Methylated DNA , 1992, Cell.

[61]  A. Bird,et al.  Identification of a mammalian protein that binds specifically to DNA containing methylated CpGs , 1989, Cell.

[62]  J. Allan,et al.  Participation of core histone "tails" in the stabilization of the chromatin solenoid , 1982, The Journal of cell biology.

[63]  A. Bird.  DNA methylation and the frequency of CpG in animal DNA. , 1980, Nucleic acids research.