MECP2 gene

Mutation of X chromosome MeCP2 gene results in neurological defects in human females (Rett syndrome) and mice. 

Three of the MBD family members, namely MeCP2, MBDl, and MBD2, have been shown to act as transcriptional repressors in vitro and in cell culture assays in vivo [86-89,75,76]. Initially it was proposed that methyl-CpG binding proteins inhibit transcription directly, by binding to methylated DNA and blocking the access of transcription factors to their sites at gene promoters. It was soon... 

Balmer D, Arredondo J, Samaco RC, LaSaUe JM (2002) MECP2 mutations in Rett syndrome adversely affect lymphocyte growth, but do not affect imprinted gene expression in blood or brain. Hum Genet 110 545-552. 

Peddada S, Yasui DH, LasaUe JM (2006) Inhibitors of differentiation (IDl, 1D2, 1D3 and 1D4) genes are neuronal targets of MeCP2 that are elevated in Rett syndrome. Hum Mol Genet 15 2003-2014. 

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