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

被引：5
|
作者：
Tokuyama, Takeshi [1 ]
Ahmed, Razan Elfadil [1 ]
Chanthra, Nawin [1 ]
Anzai, Tatsuya [1 ,2 ]
Uosaki, Hideki [1 ]
机构：
[1] Jichi Med Univ, Ctr Mol Med, Div Regenerat Med, Shimotsuke, Tochigi 3290498, Japan
[2] Jichi Med Univ, Dept Pediat, Shimotsuke, Tochigi 3290498, Japan
来源：
BIOLOGY-BASEL | 2021年 / 10卷 / 10期
关键词：
mitochondrial disease; mitochondrial cardiomyopathy; induced pluripotent stem cells (iPSC); iPSC-derived cardiomyocyte; COMPLEX-I DEFICIENCY; IPSC-DERIVED CARDIOMYOCYTES; DNA MUTATIONS; FUNCTIONAL CARDIOMYOCYTES; OXIDATIVE-PHOSPHORYLATION; FRIEDREICH ATAXIA; CRYSTAL-STRUCTURE; GENE-EXPRESSION; ASSEMBLY FACTOR; BARTH SYNDROME;
D O I：
10.3390/biology10100981
中图分类号：
Q [生物科学];
学科分类号：
07 ; 0710 ; 09 ;
摘要：
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.
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页数：21
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