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To investigate the cardiac cellular manifestation of DMD, we generated induced pluripotent stem cells (iPSCs) and iPSC\u2010derived cardiomyocytes (iPSC\u2010CMs) from two DMD patients: a male and female manifesting heterozygous carrier. Dystrophin mRNA and protein expression were analysed by qRT\u2010PCR, RNAseq, Western blot and immunofluorescence staining. For comprehensive electrophysiological analysis, current and voltage clamp were used to record transmembrane action potentials and ion currents, respectively. Microelectrode array was used to record extracellular electrograms. X\u2010inactive specific transcript (XIST) and dystrophin expression analyses revealed that female iPSCs underwent X chromosome reactivation (XCR) or erosion of X chromosome inactivation, which was maintained in female iPSC\u2010CMs displaying mixed X chromosome expression of wild type (WT) and mutated alleles. Both DMD female and male iPSC\u2010CMs presented low spontaneous firing rate, arrhythmias and prolonged action potential duration. DMD female iPSC\u2010CMs displayed increased beat rate variability (BRV). DMD male iPSC\u2010CMs manifested decreased <jats:italic>I<\/jats:italic><jats:sub>f<\/jats:sub> density, and DMD female and male iPSC\u2010CMs showed increased <jats:italic>I<\/jats:italic><jats:sub>Ca,L <\/jats:sub>density. Our findings demonstrate cellular mechanisms underlying electrophysiological abnormalities and cardiac arrhythmias in DMD.<\/jats:p>","DOI":"10.1111\/jcmm.14124","type":"journal-article","created":{"date-parts":[[2019,1,9]],"date-time":"2019-01-09T04:32:48Z","timestamp":1547008368000},"page":"2125-2135","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":41,"title":["Electrophysiological abnormalities in induced pluripotent stem cell\u2010derived cardiomyocytes generated from Duchenne muscular dystrophy patients"],"prefix":"10.1111","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2185-5054","authenticated-orcid":false,"given":"Binyamin","family":"Eisen","sequence":"first","affiliation":[{"name":"Department of Physiology, Biophysics and Systems Biology, Rappaport Faculty of Medicine Technion \u2013 Israel Institute of Technology Haifa 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