biostudies-literatureUnknown5Hara MMethyl-CpG-binding protein 2 (MeCP2) is an epigenetic regulator of gene expression that is essential for normal brain development. Mutations in MeCP2 lead to disrupted neuronal function and can cause Rett syndrome (RTT), a neurodevelopmental disorder. Previous studies reported cardiac dysfunction, including arrhythmias in both RTT patients and animal models of RTT. In addition, recent studies indicate that MeCP2 may be involved in cardiac development and dysfunction, but its role in the developing and adult heart remains unknown. In this study, we found that Mecp2-null ESCs could differentiate into cardiomyocytes, but the development and further differentiation of cardiovascular progenitors were significantly affected in MeCP2 deficiency. In addition, we revealed that loss of MeCP2 led to Scientific reports11204https://www.ebi.ac.uk/biostudies/studies/S-EPMC4466896biostudies-literatureDisturbance of cardiac gene expression and cardiomyocyte structure predisposes Mecp2-null mice to arrhythmias.PMC4466896Nakamura KTakemura GOkabe YTanaka EOkayama SYasukawa HMitsumasu CTakano MTakahashi TYamashita YMatsuishi TIgata SMinami THara MKosai KfalseDisturbance of cardiac gene expression and cardiomyocyte structure predisposes Mecp2-null mice to arrhythmias.Methyl-CpG-binding protein 2 (MeCP2) is an epigenetic regulator of gene expression that is essential for normal brain development. Mutations in MeCP2 lead to disrupted neuronal function and can cause Rett syndrome (RTT), a neurodevelopmental disorder. Previous studies reported cardiac dysfunction, including arrhythmias in both RTT patients and animal models of RTT. In addition, recent studies indicate that MeCP2 may be involved in cardiac development and dysfunction, but its role in the developing and adult heart remains unknown. In this study, we found that Mecp2-null ESCs could differentiate into cardiomyocytes, but the development and further differentiation of cardiovascular progenitors were significantly affected in MeCP2 deficiency. In addition, we revealed that loss of MeCP2 led to 2015-01-01T00:00:00Z2015 Jun2026-05-30T05:36:34.808Z2019-03-27T01:53:26ZS-EPMC44668962607355610.1038/srep11204