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Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy

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Department of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, Japan
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Department of Chemical Systems Engineering, School of Engineering, Nagoya University, Nagoya 464-8603, Japan
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Division of Biochemical Engineering, Radioisotope Research Center, Kyoto University, Kyoto 606-8501, Japan
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Graduate School of Systems Life Sciences, Kyushu University, Fukuoka 819-0395, Japan
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Center for iPS Cell Research and Application, Kyoto University, Kyoto 606-8507, Japan
*
Author to whom correspondence should be addressed.
Academic Editors: Giovanni Amabile and Jacques P. Tremblay
Cells 2021, 10(10), 2556; https://doi.org/10.3390/cells10102556
Received: 26 August 2021 / Revised: 16 September 2021 / Accepted: 25 September 2021 / Published: 27 September 2021
(This article belongs to the Special Issue Cell and Tissue Engineering for Functional Analysis)
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. View Full-Text
Keywords: Duchenne muscular dystrophy; human induced pluripotent stem cell; myotube; contractile activity; CRISPR/Cas9 Duchenne muscular dystrophy; human induced pluripotent stem cell; myotube; contractile activity; CRISPR/Cas9
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MDPI and ACS Style

Yoshioka, K.; Ito, A.; Horie, M.; Ikeda, K.; Kataoka, S.; Sato, K.; Yoshigai, T.; Sakurai, H.; Hotta, A.; Kawabe, Y.; Kamihira, M. Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy. Cells 2021, 10, 2556. https://doi.org/10.3390/cells10102556

AMA Style

Yoshioka K, Ito A, Horie M, Ikeda K, Kataoka S, Sato K, Yoshigai T, Sakurai H, Hotta A, Kawabe Y, Kamihira M. Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy. Cells. 2021; 10(10):2556. https://doi.org/10.3390/cells10102556

Chicago/Turabian Style

Yoshioka, Kantaro, Akira Ito, Masanobu Horie, Kazushi Ikeda, Sho Kataoka, Keiichiro Sato, Taichi Yoshigai, Hidetoshi Sakurai, Akitsu Hotta, Yoshinori Kawabe, and Masamichi Kamihira. 2021. "Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy" Cells 10, no. 10: 2556. https://doi.org/10.3390/cells10102556

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