Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy
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 r...
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MDPI AG
2021-09-01
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| author | Kantaro Yoshioka Akira Ito Masanobu Horie Kazushi Ikeda Sho Kataoka Keiichiro Sato Taichi Yoshigai Hidetoshi Sakurai Akitsu Hotta Yoshinori Kawabe Masamichi Kamihira |
| author_facet | Kantaro Yoshioka Akira Ito Masanobu Horie Kazushi Ikeda Sho Kataoka Keiichiro Sato Taichi Yoshigai Hidetoshi Sakurai Akitsu Hotta Yoshinori Kawabe Masamichi Kamihira |
| author_sort | Kantaro Yoshioka |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-10T06:39:16Z |
| format | Article |
| id | doaj.art-2bd0e08a180f4ed1a920fca8892aae32 |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-10T06:39:16Z |
| publishDate | 2021-09-01 |
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| record_format | Article |
| series | Cells |
| spelling | doaj.art-2bd0e08a180f4ed1a920fca8892aae322023-11-22T17:45:31ZengMDPI AGCells2073-44092021-09-011010255610.3390/cells10102556Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular DystrophyKantaro Yoshioka0Akira Ito1Masanobu Horie2Kazushi Ikeda3Sho Kataoka4Keiichiro Sato5Taichi Yoshigai6Hidetoshi Sakurai7Akitsu Hotta8Yoshinori Kawabe9Masamichi Kamihira10Department of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, JapanDepartment of Chemical Systems Engineering, School of Engineering, Nagoya University, Nagoya 464-8603, JapanDivision of Biochemical Engineering, Radioisotope Research Center, Kyoto University, Kyoto 606-8501, JapanGraduate School of Systems Life Sciences, Kyushu University, Fukuoka 819-0395, JapanDepartment of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, JapanDepartment of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, JapanDepartment of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, JapanCenter for iPS Cell Research and Application, Kyoto University, Kyoto 606-8507, JapanCenter for iPS Cell Research and Application, Kyoto University, Kyoto 606-8507, JapanDepartment of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, JapanDepartment of Chemical Engineering, Faculty of Engineering, Kyushu University, Fukuoka 819-0395, JapanDuchenne 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.https://www.mdpi.com/2073-4409/10/10/2556Duchenne muscular dystrophyhuman induced pluripotent stem cellmyotubecontractile activityCRISPR/Cas9 |
| spellingShingle | Kantaro Yoshioka Akira Ito Masanobu Horie Kazushi Ikeda Sho Kataoka Keiichiro Sato Taichi Yoshigai Hidetoshi Sakurai Akitsu Hotta Yoshinori Kawabe Masamichi Kamihira Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy Cells Duchenne muscular dystrophy human induced pluripotent stem cell myotube contractile activity CRISPR/Cas9 |
| title | Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy |
| title_full | Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy |
| title_fullStr | Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy |
| title_full_unstemmed | Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy |
| title_short | Contractile Activity of Myotubes Derived from Human Induced Pluripotent Stem Cells: A Model of Duchenne Muscular Dystrophy |
| title_sort | contractile activity of myotubes derived from human induced pluripotent stem cells a model of duchenne muscular dystrophy |
| topic | Duchenne muscular dystrophy human induced pluripotent stem cell myotube contractile activity CRISPR/Cas9 |
| url | https://www.mdpi.com/2073-4409/10/10/2556 |
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