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Review

Disease Modeling of Mitochondrial Cardiomyopathy Using Patient-Specific Induced Pluripotent Stem Cells

1
Division of Regenerative Medicine, Center for Molecular Medicine, Jichi Medical University, Shimotsuke 329-0498, Japan
2
Department of Pediatrics, Jichi Medical University, Shimotsuke 329-0498, Japan
*
Author to whom correspondence should be addressed.
Academic Editors: Toshio Nakanishi and Nanako Kawaguchi
Biology 2021, 10(10), 981; https://doi.org/10.3390/biology10100981
Received: 31 August 2021 / Revised: 25 September 2021 / Accepted: 26 September 2021 / Published: 29 September 2021
(This article belongs to the Special Issue Stem Cells for Cardiovascular Biology and Medicine)
Mitochondria are essential intracellular organelles that generate energy within the cell. Mitochondria are present in all organs, and organs are powered by the energy produced by mitochondria. Mitochondria are composed of proteins encoded by nuclear and mitochondrial DNA. It is possible that mutations in nuclear and mitochondrial DNA cause alterations in proteins that make up mitochondria, resulting in mitochondrial dysfunction. Since cellular and organ functions depend on mitochondrial function, this mitochondrial dysfunction can lead to tissue dysfunction, namely mitochondrial diseases. In recent years, there have been many reports of the multifaceted functions of mitochondria. However, there is still little knowledge about the diseases. This problem arises because there is no suitable model to mimic mitochondrial diseases. In this paper, we introduce mitochondrial cardiomyopathy models that mimic patients’ cardiomyocytes using human induced pluripotent stem cells (iPSCs). The use of human iPSCs will advance the understanding of the pathogenesis of mitochondrial cardiomyopathy and the development of new drugs.
Mitochondrial cardiomyopathy (MCM) is characterized as an oxidative phosphorylation disorder of the heart. More than 100 genetic variants in nuclear or mitochondrial DNA have been associated with MCM. However, the underlying molecular mechanisms linking genetic variants to MCM are not fully understood due to the lack of appropriate cellular and animal models. Patient-specific induced pluripotent stem cell (iPSC)-derived cardiomyocytes (iPSC-CMs) provide an attractive experimental platform for modeling cardiovascular diseases and predicting drug efficacy to such diseases. Here we introduce the pathological and therapeutic studies of MCM using iPSC-CMs and discuss the questions and latest strategies for research using iPSC-CMs. View Full-Text
Keywords: mitochondrial disease; mitochondrial cardiomyopathy; induced pluripotent stem cells (iPSC); iPSC-derived cardiomyocyte mitochondrial disease; mitochondrial cardiomyopathy; induced pluripotent stem cells (iPSC); iPSC-derived cardiomyocyte
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MDPI and ACS Style

Tokuyama, T.; Ahmed, R.E.; Chanthra, N.; Anzai, T.; Uosaki, H. Disease Modeling of Mitochondrial Cardiomyopathy Using Patient-Specific Induced Pluripotent Stem Cells. Biology 2021, 10, 981. https://doi.org/10.3390/biology10100981

AMA Style

Tokuyama T, Ahmed RE, Chanthra N, Anzai T, Uosaki H. Disease Modeling of Mitochondrial Cardiomyopathy Using Patient-Specific Induced Pluripotent Stem Cells. Biology. 2021; 10(10):981. https://doi.org/10.3390/biology10100981

Chicago/Turabian Style

Tokuyama, Takeshi, Razan E. Ahmed, Nawin Chanthra, Tatsuya Anzai, and Hideki Uosaki. 2021. "Disease Modeling of Mitochondrial Cardiomyopathy Using Patient-Specific Induced Pluripotent Stem Cells" Biology 10, no. 10: 981. https://doi.org/10.3390/biology10100981

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