The Odyssey of MeCP2 and Parental Imprinting

DNA methylation in mammals has long been implicated in the epigenetic mechanism of parentalimprinting, in which selective expression of one allele of specific genes is based on parentalorigin. Methyl CpG binding protein 2 (MeCP2) selectively binds to methylated DNA andmutations in the MECP2 cause the autism-spectrum neurodevelopmental disorder Rettsyndrome. This review outlines the emerging story of how MeCP2 has been implicated in theregulation of specific imprinted genes and loci, including UBE3A and DLX5. The story ofMeCP2 and parental imprinting has unfolded with some interesting but unexpected twists,revealing new insights on the function of MeCP2 in the process.