MECP2 encodes a protein that binds to methylated DNA. MeCP2 protein bind methylated DNA and repress transcription.

Pathology

 mutations of MECP2 in Rett syndrome

• abnormal gene expression patterns within the first year of life.
• Girls with Rett syndrome display reduced brain growth, loss of developmental milestones and profound mental disabilities.

See also

 DNA methylation
 methyl-DNA-binding proteins

References

 Caballero IM, Hendrich B. MeCP2 in neurons: closing in on the causes of Rett syndrome. Hum Mol Genet. 2005 Apr 15;14 Spec No 1:R19-26. PMID: #15809268#

 Kriaucionis S, Bird A. DNA methylation and Rett syndrome. Hum Mol Genet. 2003 Oct 15;12 Spec No 2:R221-7. Epub 2003 Aug 19. PMID: #12928486#

 Dragich J, Houwink-Manville I, Schanen C. Rett syndrome: a surprising result of mutation in MECP2. Hum Mol Genet. 2000 Oct;9(16):2365-75. PMID: #11005791#