MeCP2 Repression Goes Nonglobal

Abstract

Methylation of CpG islands in gene promoters results in silencing of those genes. Mutation of a methyl-CpG binding domain protein called MeCP2 that contributes to the maintenance of methylation-mediated gene silencing is associated with a severe neurological disease called Rett syndrome. As Klose and Bird report in their Perspective, new work published here (Chen et al., Martinowich et al.) and elsewhere demonstrates that MeCP2 normally represses the expression of two genes-BDNF in rat and Hairy2a in frog-that are crucial for normal development of the nervous system.