Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice

  title={Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice},
  author={R. Y. Chen and S. Akbarian and Matthew Tudor and R. Jaenisch},
  journal={Nature Genetics},
  • R. Y. Chen, S. Akbarian, +1 author R. Jaenisch
  • Published 2001
  • Biology, Medicine
  • Nature Genetics
  • Mecp2 is an X-linked gene encoding a nuclear protein that binds specifically to methylated DNA (ref. 1) and functions as a general transcriptional repressor by associating with chromatin-remodeling complexes. Mecp2 is expressed at high levels in the postnatal brain, indicating that methylation-dependent regulation of gene expression may have a crucial role in the mammalian central nervous system. Consistent with this notion is the recent demonstration that MECP2 mutations cause Rett syndrome… CONTINUE READING
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