The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

Mecp2  -  methyl CpG binding protein 2

Mus musculus

Synonyms: 1500041B07Rik, BB130002, D630021H01Rik, Mbd5, MeCp-2 protein, ...
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of Mecp2

  • Our findings raise the possibility that mitochondrial dysfunction contributes to pathology of the Mecp2-null mouse and may contribute to the long-known resemblance between Rett syndrome and certain mitochondrial disorders [1].
  • However, marked respiratory depression following hypoxic hyperventilation was only seen in Mecp2+/- animals [2].
  • Selective deletion of Mecp2 in post-mitotic neurons in mice results in a Rett-like phenotype characterized by disturbances in motor activity and body weight, suggesting that these symptoms are exclusively caused by neuronal deficiency [2].
  • Both female mice heterozygous for a null mutation in Mecp2 (Mecp2+/-) and those with selective deletion of the protein in neurons (Mecp2+/nestin-Cre lox), showed an initial response to hypoxia that exceeded that in wild type (WT) [2].
  • We show that Brm and MeCP2 assembly on chromatin occurs on methylated genes in cancer and the gene FMR1 in fragile X syndrome [3].

Psychiatry related information on Mecp2


High impact information on Mecp2

  • Transcriptional repression of methylated genes can be mediated by the methyl-CpG binding protein MeCP2 [3].
  • Brahma links the SWI/SNF chromatin-remodeling complex with MeCP2-dependent transcriptional silencing [3].
  • In some genes, the level of expression is adjusted further in neurons by CoREST/MeCP2 repressor complexes that remain bound to a site of methylated DNA distinct from the RE1 site [8].
  • A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome [9].
  • Brain-specific deletion of Mecp2 at embryonic day (E) 12 resulted in a phenotype identical to that of the null mutation, indicating that the phenotype is caused by Mecp2 deficiency in the CNS rather than in peripheral tissues [10].

Chemical compound and disease context of Mecp2

  • These data suggest hypotheses concerning 5-HT modulation of vagal function for testing in MeCP2 knockout mice to understand mechanisms underlying autonomic dysfunction in patients with Rett syndrome [11].
  • Rett syndrome is caused by loss-of-function mutations in the gene encoding the methyl DNA-binding factor MeCP2 [12].

Biological context of Mecp2


Anatomical context of Mecp2


Associations of Mecp2 with chemical compounds


Physical interactions of Mecp2


Other interactions of Mecp2

  • MeCP2-induced rearrangement of heterochromatin occurred throughout interphase, did not depend on the H3K9 histone methylation pathway, and required the methyl CpG-binding domain (MBD) only [24].
  • Therefore, it is likely that the number of precursor cells allocated to the Purkinje cell lineage is affected by a paternally inherited mutation in Mecp2 [25].
  • In vivo analysis of the olfactory system demonstrates that Mecp2 deficiency leads to a transient delay in the terminal differentiation of olfactory neurons [26].
  • However, the expression of the methyl-CpG-binding protein MeCP2 is greatly reduced in Sp1-/- embryos [27].
  • Uqcrc1 encodes a subunit of mitochondrial respiratory complex III, and isolated mitochondria from the Mecp2-null brain showed elevated respiration rates associated with respiratory complex III and an overall reduction in coupling [1].

Analytical, diagnostic and therapeutic context of Mecp2

  • As Ube3a is only imprinted in brain, we evaluated Ube3a expression in brains of 15 different litters of neonatal or 8-week-old male Mecp2 mutant mice by real-time quantitative RT-PCR and western blot analysis [28].
  • By comparative sequence analysis, we identified a previously unknown, non-coding 5' exon embedded in a CpG island associated with MECP2/Mecp2 [14].
  • Real-time RT-PCR measurements of Mecp2 transcript levels showed variations with mouse age in two distinctive patterns that are unique to the central nervous system and the visceral organs, respectively [29].
  • To clarify whether Mecp2 dysfunction may cause impairment of the monoaminergic and serotonergic systems, we measured the whole brain concentrations of biogenic amines and related substrates in three mecp2-null male mice and four control mice of each age at 0-42 postnatal days by HPLC methods [30].
  • All four ID proteins were significantly increased in Mecp2-deficient mouse and human RTT brain using immunofluorescence and laser scanning cytometric analyses [31].


  1. Gene expression analysis exposes mitochondrial abnormalities in a mouse model of Rett syndrome. Kriaucionis, S., Paterson, A., Curtis, J., Guy, J., Macleod, N., Bird, A. Mol. Cell. Biol. (2006) [Pubmed]
  2. Separate respiratory phenotypes in methyl-CpG-binding protein 2 (Mecp2) deficient mice. Bissonnette, J.M., Knopp, S.J. Pediatr. Res. (2006) [Pubmed]
  3. Brahma links the SWI/SNF chromatin-remodeling complex with MeCP2-dependent transcriptional silencing. Harikrishnan, K.N., Chow, M.Z., Baker, E.K., Pal, S., Bassal, S., Brasacchio, D., Wang, L., Craig, J.M., Jones, P.L., Sif, S., El-Osta, A. Nat. Genet. (2005) [Pubmed]
  4. An altered neonatal behavioral phenotype in Mecp2 mutant mice. Picker, J.D., Yang, R., Ricceri, L., Berger-Sweeney, J. Neuroreport (2006) [Pubmed]
  5. Delayed maturation of neuronal architecture and synaptogenesis in cerebral cortex of Mecp2-deficient mice. Fukuda, T., Itoh, M., Ichikawa, T., Washiyama, K., Goto, Y. J. Neuropathol. Exp. Neurol. (2005) [Pubmed]
  6. Insight into Rett syndrome: MeCP2 levels display tissue- and cell-specific differences and correlate with neuronal maturation. Shahbazian, M.D., Antalffy, B., Armstrong, D.L., Zoghbi, H.Y. Hum. Mol. Genet. (2002) [Pubmed]
  7. MeCP2 dysfunction in humans and mice. Zoghbi, H.Y. J. Child Neurol. (2005) [Pubmed]
  8. REST and its corepressors mediate plasticity of neuronal gene chromatin throughout neurogenesis. Ballas, N., Grunseich, C., Lu, D.D., Speh, J.C., Mandel, G. Cell (2005) [Pubmed]
  9. A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome. Guy, J., Hendrich, B., Holmes, M., Martin, J.E., Bird, A. Nat. Genet. (2001) [Pubmed]
  10. Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice. Chen, R.Z., Akbarian, S., Tudor, M., Jaenisch, R. Nat. Genet. (2001) [Pubmed]
  11. Serotonin transporter abnormality in the dorsal motor nucleus of the vagus in Rett syndrome: potential implications for clinical autonomic dysfunction. Paterson, D.S., Thompson, E.G., Belliveau, R.A., Antalffy, B.A., Trachtenberg, F.L., Armstrong, D.D., Kinney, H.C. J. Neuropathol. Exp. Neurol. (2005) [Pubmed]
  12. Increased dendritic complexity and axonal length in cultured mouse cortical neurons overexpressing methyl-CpG-binding protein MeCP2. Jugloff, D.G., Jung, B.P., Purushotham, D., Logan, R., Eubanks, J.H. Neurobiol. Dis. (2005) [Pubmed]
  13. Genetic and physical mapping of a gene encoding a methyl CpG binding protein, Mecp2, to the mouse X chromosome. Quaderi, N.A., Meehan, R.R., Tate, P.H., Cross, S.H., Bird, A.P., Chatterjee, A., Herman, G.E., Brown, S.D. Genomics (1994) [Pubmed]
  14. Comparative sequence analysis of the MECP2-locus in human and mouse reveals new transcribed regions. Reichwald, K., Thiesen, J., Wiehe, T., Weitzel, J., Poustka, W.A., Rosenthal, A., Platzer, M., Strätling, W.H., Kioschis, P. Mamm. Genome (2000) [Pubmed]
  15. Mecp2 deficiency disrupts norepinephrine and respiratory systems in mice. Viemari, J.C., Roux, J.C., Tryba, A.K., Saywell, V., Burnet, H., Peña, F., Zanella, S., Bévengut, M., Barthelemy-Requin, M., Herzing, L.B., Moncla, A., Mancini, J., Ramirez, J.M., Villard, L., Hilaire, G. J. Neurosci. (2005) [Pubmed]
  16. Dysregulation of brain-derived neurotrophic factor expression and neurosecretory function in Mecp2 null mice. Wang, H., Chan, S.A., Ogier, M., Hellard, D., Wang, Q., Smith, C., Katz, D.M. J. Neurosci. (2006) [Pubmed]
  17. A complex pattern of evolutionary conservation and alternative polyadenylation within the long 3"-untranslated region of the methyl-CpG-binding protein 2 gene (MeCP2) suggests a regulatory role in gene expression. Coy, J.F., Sedlacek, Z., Bächner, D., Delius, H., Poustka, A. Hum. Mol. Genet. (1999) [Pubmed]
  18. A segment of the Mecp2 promoter is sufficient to drive expression in neurons. Adachi, M., Keefer, E.W., Jones, F.S. Hum. Mol. Genet. (2005) [Pubmed]
  19. Inhibitors of histone deacetylase and DNA methyltransferase synergistically activate the methylated metallothionein I promoter by activating the transcription factor MTF-1 and forming an open chromatin structure. Ghoshal, K., Datta, J., Majumder, S., Bai, S., Dong, X., Parthun, M., Jacob, S.T. Mol. Cell. Biol. (2002) [Pubmed]
  20. The methyl-CpG-binding protein MeCP2 links DNA methylation to histone methylation. Fuks, F., Hurd, P.J., Wolf, D., Nan, X., Bird, A.P., Kouzarides, T. J. Biol. Chem. (2003) [Pubmed]
  21. FBP WW domains and the Abl SH3 domain bind to a specific class of proline-rich ligands. Bedford, M.T., Chan, D.C., Leder, P. EMBO J. (1997) [Pubmed]
  22. Dissection of the methyl-CpG binding domain from the chromosomal protein MeCP2. Nan, X., Meehan, R.R., Bird, A. Nucleic Acids Res. (1993) [Pubmed]
  23. Up-regulation of glucocorticoid-regulated genes in a mouse model of Rett syndrome. Nuber, U.A., Kriaucionis, S., Roloff, T.C., Guy, J., Selfridge, J., Steinhoff, C., Schulz, R., Lipkowitz, B., Ropers, H.H., Holmes, M.C., Bird, A. Hum. Mol. Genet. (2005) [Pubmed]
  24. Methyl CpG-binding proteins induce large-scale chromatin reorganization during terminal differentiation. Brero, A., Easwaran, H.P., Nowak, D., Grunewald, I., Cremer, T., Leonhardt, H., Cardoso, M.C. J. Cell Biol. (2005) [Pubmed]
  25. Reduced proportion of Purkinje cells expressing paternally derived mutant Mecp2308 allele in female mouse cerebellum is not due to a skewed primary pattern of X-chromosome inactivation. Watson, C.M., Pelka, G.J., Radziewic, T., Shahbazian, M.D., Christodoulou, J., Williamson, S.L., Tam, P.P. Hum. Mol. Genet. (2005) [Pubmed]
  26. The transcriptional repressor Mecp2 regulates terminal neuronal differentiation. Matarazzo, V., Cohen, D., Palmer, A.M., Simpson, P.J., Khokhar, B., Pan, S.J., Ronnett, G.V. Mol. Cell. Neurosci. (2004) [Pubmed]
  27. Transcription factor Sp1 is essential for early embryonic development but dispensable for cell growth and differentiation. Marin, M., Karis, A., Visser, P., Grosveld, F., Philipsen, S. Cell (1997) [Pubmed]
  28. Ube3a expression is not altered in Mecp2 mutant mice. Jordan, C., Francke, U. Hum. Mol. Genet. (2006) [Pubmed]
  29. Distinct expression profiles of Mecp2 transcripts with different lengths of 3'UTR in the brain and visceral organs during mouse development. Pelka, G.J., Watson, C.M., Christodoulou, J., Tam, P.P. Genomics (2005) [Pubmed]
  30. Defect in normal developmental increase of the brain biogenic amine concentrations in the mecp2-null mouse. Ide, S., Itoh, M., Goto, Y. Neurosci. Lett. (2005) [Pubmed]
  31. Inhibitors of differentiation (ID1, ID2, ID3 and ID4) genes are neuronal targets of MeCP2 that are elevated in Rett syndrome. Peddada, S., Yasui, D.H., LaSalle, J.M. Hum. Mol. Genet. (2006) [Pubmed]
WikiGenes - Universities