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Science 302 (5646): 793-795

Copyright © 2003 by the American Association for the Advancement of Science

MeCP2 Repression Goes Nonglobal

Robert Klose and Adrian Bird

Methylation of CpG islands in gene promoters results in silencing of those genes. Mutation of a methyl-CpG binding domain protein called MeCP2 that contributes to the maintenance of methylation-mediated gene silencing is associated with a severe neurological disease called Rett syndrome. As Klose and Bird report in their Perspective, new work published here (Chen et al., Martinowich et al.) and elsewhere demonstrates that MeCP2 normally represses the expression of two genes-BDNF in rat and Hairy2a in frog-that are crucial for normal development of the nervous system.

The authors are at the Wellcome Trust Centre for Cell Biology, University of Edinburgh, The King's Buildings, Edinburgh EH9 3JR, UK. E-mail: a.bird{at}

MeCP2: a novel Huntingtin interactor.
K. N. McFarland, M. N. Huizenga, S. B. Darnell, G. R. Sangrey, O. Berezovska, J.-H. J. Cha, T. F. Outeiro, and G. Sadri-Vakili (2014)
Hum. Mol. Genet. 23, 1036-1044
   Abstract »    Full Text »    PDF »
The MeCP2/YY1 interaction regulates ANT1 expression at 4q35: novel hints for Rett syndrome pathogenesis.
G. Forlani, E. Giarda, U. Ala, F. Di Cunto, M. Salani, R. Tupler, C. Kilstrup-Nielsen, and N. Landsberger (2010)
Hum. Mol. Genet. 19, 3114-3123
   Abstract »    Full Text »    PDF »
Regulation of heterochromatin remodelling and myogenin expression during muscle differentiation by FAK interaction with MBD2.
S.-W. Luo, C. Zhang, B. Zhang, C.-H. Kim, Y.-Z. Qiu, Q.-S. Du, L. Mei, and W.-C. Xiong (2009)
EMBO J. 28, 2568-2582
   Abstract »    Full Text »    PDF »
The loss of methyl-CpG binding protein 1 leads to autism-like behavioral deficits.
A. M. Allan, X. Liang, Y. Luo, C. Pak, X. Li, K. E. Szulwach, D. Chen, P. Jin, and X. Zhao (2008)
Hum. Mol. Genet. 17, 2047-2057
   Abstract »    Full Text »    PDF »
Transcriptional and posttranscriptional regulation of endothelial nitric oxide synthase expression.
C. D. Searles (2006)
Am J Physiol Cell Physiol 291, C803-C816
   Abstract »    Full Text »    PDF »
Reelin and glutamic acid decarboxylase67 promoter remodeling in an epigenetic methionine-induced mouse model of schizophrenia.
E. Dong, R. C. Agis-Balboa, M. V. Simonini, D. R. Grayson, E. Costa, and A. Guidotti (2005)
PNAS 102, 12578-12583
   Abstract »    Full Text »    PDF »
T-bet antagonizes mSin3a recruitment and transactivates a fully methylated IFN-{gamma} promoter via a conserved T-box half-site.
Y. Tong, T. Aune, and M. Boothby (2005)
PNAS 102, 2034-2039
   Abstract »    Full Text »    PDF »
Rett syndrome: clinical review and genetic update.
L S Weaving, C J Ellaway, J Gecz, and J Christodoulou (2005)
J. Med. Genet. 42, 1-7
   Abstract »    Full Text »    PDF »
Components of the DNA Methylation System of Chromatin Control Are RNA-binding Proteins.
L. Jeffery and S. Nakielny (2004)
J. Biol. Chem. 279, 49479-49487
   Abstract »    Full Text »    PDF »
MeCP2 Behaves as an Elongated Monomer That Does Not Stably Associate with the Sin3a Chromatin Remodeling Complex.
R. J. Klose and A. P. Bird (2004)
J. Biol. Chem. 279, 46490-46496
   Abstract »    Full Text »    PDF »
The Cell-specific Expression of Endothelial Nitric-oxide Synthase: A ROLE FOR DNA METHYLATION.
Y. Chan, J. E. Fish, C. D'Abreo, S. Lin, G. B. Robb, A.-M. Teichert, F. Karantzoulis-Fegaras, A. Keightley, B. M. Steer, and P. A. Marsden (2004)
J. Biol. Chem. 279, 35087-35100
   Abstract »    Full Text »    PDF »

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