{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T19:15:16Z","timestamp":1790536516547,"version":"4.1.0"},"reference-count":34,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2021,9,27]],"date-time":"2021-09-27T00:00:00Z","timestamp":1632700800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["20H00322"],"award-info":[{"award-number":["20H00322"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Duchenne muscular dystrophy (DMD) is a genetic disorder that results from deficiency of the dystrophin protein. In recent years, DMD pathological models have been created using induced pluripotent stem (iPS) cells derived from DMD patients. In addition, gene therapy using CRISPR-Cas9 technology to repair the dystrophin gene has been proposed as a new treatment method for DMD. However, it is not known whether the contractile function of myotubes derived from gene-repaired iPS cells can be restored. We therefore investigated the maturation of myotubes in electrical pulse stimulation culture and examined the effect of gene repair by observing the contractile behaviour of myotubes. The contraction activity of myotubes derived from dystrophin-gene repaired iPS cells was improved by electrical pulse stimulation culture. The iPS cell method used in this study for evaluating muscle contractile activity is a useful technique for analysing the mechanism of hereditary muscular disease pathogenesis and for evaluating the efficacy of new drugs and gene therapy.<\/jats:p>","DOI":"10.3390\/cells10102556","type":"journal-article","created":{"date-parts":[[2021,9,27]],"date-time":"2021-09-27T10:39:20Z","timestamp":1632739160000},"page":"2556","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy"],"prefix":"10.3390","volume":"10","author":[{"given":"Kantaro","family":"Yoshioka","sequence":"first","affiliation":[{"name":"Department of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akira","family":"Ito","sequence":"additional","affiliation":[{"name":"Department of Chemical Systems Engineering, School of Engineering, Nagoya University, Nagoya 464-8603, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9795-2582","authenticated-orcid":false,"given":"Masanobu","family":"Horie","sequence":"additional","affiliation":[{"name":"Division of Biochemical Engineering, Radioisotope Research Center, Kyoto University, Kyoto 606-8501, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kazushi","family":"Ikeda","sequence":"additional","affiliation":[{"name":"Graduate School of Systems Life Sciences, Kyushu University, Fukuoka 819-0395, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sho","family":"Kataoka","sequence":"additional","affiliation":[{"name":"Department of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keiichiro","family":"Sato","sequence":"additional","affiliation":[{"name":"Department of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taichi","family":"Yoshigai","sequence":"additional","affiliation":[{"name":"Department of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hidetoshi","family":"Sakurai","sequence":"additional","affiliation":[{"name":"Center for iPS Cell Research and Application, Kyoto University, Kyoto 606-8507, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2619-7441","authenticated-orcid":false,"given":"Akitsu","family":"Hotta","sequence":"additional","affiliation":[{"name":"Center for iPS Cell Research and Application, Kyoto University, Kyoto 606-8507, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshinori","family":"Kawabe","sequence":"additional","affiliation":[{"name":"Department of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2570-5712","authenticated-orcid":false,"given":"Masamichi","family":"Kamihira","sequence":"additional","affiliation":[{"name":"Department of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, Japan"},{"name":"Graduate School of Systems Life Sciences, Kyushu University, Fukuoka 819-0395, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,9,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1016\/S1474-4422(18)30024-3","article-title":"Diagnosis and management of Duchenne muscular dystrophy, part 1: Diagnosis, and neuromuscular, rehabilitation, endocrine, and gastrointestinal and nutritional management","volume":"17","author":"Birnkrant","year":"2018","journal-title":"Lancet Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"4585","DOI":"10.1007\/s00018-013-1396-z","article-title":"Ongoing therapeutic trials and outcome measures for Duchenne muscular dystrophy","volume":"70","author":"Govoni","year":"2013","journal-title":"Cell. 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