| First Author | Lyst MJ | Year | 2013 |
| Journal | Nat Neurosci | Volume | 16 |
| Issue | 7 | Pages | 898-902 |
| PubMed ID | 23770565 | Mgi Jnum | J:203745 |
| Mgi Id | MGI:5528617 | Doi | 10.1038/nn.3434 |
| Citation | Lyst MJ, et al. (2013) Rett syndrome mutations abolish the interaction of MeCP2 with the NCoR/SMRT co-repressor. Nat Neurosci 16(7):898-902 |
| abstractText | 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 missense RTT mutation in this domain exhibited severe RTT-like phenotypes. Our data are compatible with the hypothesis that brain dysfunction in RTT is caused by a loss of the MeCP2 'bridge' between the NCoR/SMRT co-repressors and chromatin. |
