InterPro domain: IPR045138

Methyl-CpG-binding domain protein 4 (MBD4) is a mismatch-specific DNA N-glycosylase involved in DNA repair. It has thymine glycosylase activity and is specific for G:T mismatches within methylated and unmethylated CpG sites [ 1 , 2 ].

Methyl-CpG-binding protein 2 (Mecp2) binds specifically to methylated DNA and represses transcription through the recruitment of chromatin remodeling complexes containing histone deacetylase activities [ 3 , 4 ]. It is a multi-functional protein whose function depends on its protein partners and post-translational modifications [ 5 ]. Mecp2 is required for proper mitotic spindle organisation [ 6 ] and affects proliferation and apoptosis [ 7 ]. Mutations in MeCP2 gene cause an X-linked neurodevelopmental disease known as Rett syndrome [ 8 ].

1. Identification and characterization of a family of mammalian methyl-CpG binding proteins. Mol. Cell. Biol. 18, 6538-47
2. Biphasic kinetics of the human DNA repair protein MED1 (MBD4), a mismatch-specific DNA N-glycosylase. J. Biol. Chem.
3. Methylated DNA and MeCP2 recruit histone deacetylase to repress transcription. Nat. Genet. 8, 236
4. Transcriptional repression by the methyl-CpG-binding protein MeCP2 involves a histone deacetylase complex. Nature 393, 386-9
5. MeCP2 post-translational modifications: a mechanism to control its involvement in synaptic plasticity and homeostasis? Front Cell Neurosci 35, 11819-27
6. The methyl-CpG binding protein 2 (MeCP2) localizes at the centrosome and is required for proper mitotic spindle organization. J. Biol. Chem. 19, 187-91
7. Epigenetic silencing of methyl-CpG-binding protein 2 gene affects proliferation, invasion, migration, and apoptosis of human osteosarcoma cells. Tumour Biol. 23 Suppl 1, S32-7
8. The biological functions of the methyl-CpG-binding protein MeCP2 and its implication in Rett syndrome. Brain Dev. 275, 32422-9