Anti-MeCP2 from rabbit, purified by affinity chromatography

Methyl-CpG-binding protein 2 (UniProt P51608; also known as MeCp-2 protein, MeCp2) is encoded by the MECP2 (also known as AS, MRXS13, MRXSL, PPMX, RTT, AUTSX3) gene (Gene ID 4204) in human. Methyl-CpG-binding proteins that are involved in gene expression regulation by targeting methylated DNA residues in gene promoters via a conserved methyl-CpG-binding domain (MBD; a.a. 90-162 of human MeCp2). Upon binding methylated CpG sites on the DNA strands, transcriptional repression domain (TRD; a.a. 207-492) then mediates association with SIN3A to recruit histone deacetylases (HDAC). The TRD region also contains a nuclear localization signal (NLS) sequence (a.a. 255-271) and a nuclear receptor co-repressor 2/NCoR2-interacting site (a.a. 269-309). MeCp2 protein is found in all cells in the body, including the brain, where it is found in high concentrations in neurons and is associated with maturation of the central nervous system (CNS) and in forming synaptic contacts. In contrast to other MBD family members, MECP2 is X-linked and subject to X chromosome inactivation. MECP2 gene mutations are linked to Rett syndrome (RTT), a progressive neurologic developmental disorder and one of the most common causes of mental retardation in females.