Rett syndrome mutations abolish the interaction of MeCP2 with the NCoR/SMRT co-repressor

  title={Rett syndrome mutations abolish the interaction of MeCP2 with the NCoR/SMRT co-repressor},
  author={Matthew J. Lyst and Robert Ekiert and Daniel H. Ebert and Cara Merusi and Jakub Nowak and Jim Selfridge and Jacky Guy and Nathaniel R. Kastan and Nathaniel D. Robinson and Fl{\'a}via de Lima Alves and Juri Rappsilber and Michael E. Greenberg and Adrian Bird},
  journal={Nature Neuroscience},
Rett syndrome (RTT) is a severe neurological disorder that is caused by mutations in the MECP2 gene. Many missense mutations causing RTT are clustered in the DNA-binding domain of MeCP2, suggesting that association with chromatin is critical for its function. We identified a second mutational cluster in a previously uncharacterized region of MeCP2. We found that RTT mutations in this region abolished the interaction between MeCP2 and the NCoR/SMRT co-repressor complexes. Mice bearing a common… CONTINUE READING
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structure and function

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  • Mol. Cell Endocrinol. 348, 440–449
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