MECP2 (Methyl-CpG Binding Protein 2) is a chromosomal protein that binds to methylated DNA. It can bind specifically to a single methyl-CpG pair and is not influenced by sequences flanking the methyl-CpGs. MECP2 has been shown to mediate transcriptional repression through interaction with histone deacetylase and the corepressor SIN3A. Defects in MECP2 are the cause of Rett syndrome (RTT). RTT is an X-linked dominant disease, it is a progressive neurologic developmental disorder and one of the most common causes of mental retardation in females. Recent studies have reported a new phosphorylation site at Ser80. Phosphorylation and dephosphorylation of this site may be involved in modulating the dynamic function of MECP2 in neurons transiting between resting and active states within neural circuits that underlie behaviors. (Tao et al., 2009)
