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We developed a disease model using iPSC-derived cardiomyocytes (iPSC-CMs) from a healthy individual and two DM1 patients with different CTG repeats lengths and clinical history (DM1-1300 and DM1-300). We confirmed the presence of toxic RNA foci and mis-spliced <jats:italic>MBNL1\/2<\/jats:italic> transcripts in DM1 iPSC-CMs. In DM1-1300, we identified a switch in the cardiac sodium channel <jats:italic>SCN5A<\/jats:italic> from the adult to the neonatal isoform. The down-regulation of adult <jats:italic>SCN5A<\/jats:italic> isoforms is consistent with a shift in the sodium current activation to depolarized potentials observed in DM1-1300. L-type calcium current density was higher in iPSC-CMs from DM1-1300, which is correlated with the overexpression of the Ca<jats:sub>V<\/jats:sub>1.2 transcript and proteins. Importantly, I<jats:sub>Na<\/jats:sub> and I<jats:sub>CaL<\/jats:sub> dysfunctions resulted in prolonged action potentials duration, slower velocities, and decreased overshoots. Optical mapping analysis revealed a slower conduction velocity in DM1-1300 iPSC-CM monolayers. In conclusion, our data revealed two distinct ions channels perturbations in DM1 iPSC-CM from the patient with cardiac dysfunction, one affecting Na<jats:sup>+<\/jats:sup> channels and one affecting Ca<jats:sup>2+<\/jats:sup> channels. Both have an impact on cardiac APs and ultimately on heart conduction.<\/jats:p>","DOI":"10.1038\/s41598-021-82007-8","type":"journal-article","created":{"date-parts":[[2021,1,28]],"date-time":"2021-01-28T11:04:39Z","timestamp":1611831879000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["iPSC-derived cardiomyocytes from patients with myotonic dystrophy type 1 have abnormal ion channel functions and slower conduction velocities"],"prefix":"10.1038","volume":"11","author":[{"given":"Hugo","family":"Poulin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aur\u00e9lie","family":"Mercier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammed","family":"Djemai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Val\u00e9rie","family":"Pouliot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Deschenes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed","family":"Boutjdir","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jack","family":"Puymirat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed","family":"Chahine","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,1,28]]},"reference":[{"key":"82007_CR1","doi-asserted-by":"publisher","first-page":"58","DOI":"10.1007\/BF01671719","volume":"37","author":"H Steinert","year":"1909","unstructured":"Steinert, H. 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