The Odyssey of MeCP2 and parental imprinting.
DNA methylation in mammals has long been implicated in the epigenetic mechanism of parental imprinting, in which selective expression of one allele of specific genes is based on parental origin. Methyl CpG binding protein 2 (MeCP2) selectively binds to methylated DNA and mutations in the MECP2 cause the autism-spectrum neurodevelopmental disorder Rett syndrome. This review outlines the emerging story of how MeCP2 has been implicated in the regulation of specific imprinted genes and loci, including UBE3A and DLX5. The story of MeCP2 and parental imprinting has unfolded with some interesting but unexpected twists, revealing new insights on the function of MeCP2 in the process.
LaSalle
Janine M
JM
Medical Microbiology and Immunology, One Shields Avenue, Davis, California 95616, USA. jmlasalle@ucdavis.edu
D000328:Adult; D002648:Child; D004247:DNA / Q000235:genetics; D019175:DNA Methylation; D015226:Dinucleoside Phosphates / Q000235:genetics; D005260:Female; D018392:Genomic Imprinting; D006657:Histones / Q000235:genetics / Q000378:metabolism; D006801:Humans; D008297:Male; D051783:Methyl-CpG-Binding Protein 2 / Q000235:genetics*; D009154:Mutation; D009420:Nervous System / Q000254:growth & development; D015518:Rett Syndrome / Q000235:genetics*
D004247:DNA; D015226:Dinucleoside Phosphates; D006657:Histones; D051783:Methyl-CpG-Binding Protein 2; C497392:MECP2 protein, human; C015772:CpG dinucleotide