Human induced pluripotent stem cells for studying mitochondrial diseases in the heart

被引:6
作者
Caudal, Arianne [1 ,2 ,3 ]
Ren, Lu [1 ,2 ,3 ]
Tu, Chengyi [1 ,2 ,3 ]
Wu, Joseph C. [1 ,2 ,3 ]
机构
[1] Stanford Univ, Sch Med, Stanford Cardiovasc Inst, 265 Campus Dr,G1120B, Stanford, CA 94305 USA
[2] Stanford Univ, Sch Med, Inst Stem Cell Biol & Regenerat Med, Stanford, CA 94305 USA
[3] Stanford Univ, Sch Med, Dept Med, Div Cardiol, Stanford, CA 94305 USA
基金
美国国家卫生研究院;
关键词
cardiomyocyte; cardiovascular disease; heart; iPSC; mitochondria; stem cell; HYPERTROPHIC CARDIOMYOPATHY; CARDIAC METABOLISM; BARTH-SYNDROME; CARDIOMYOCYTES; DYSFUNCTION; MATURATION; MORBIDITY; MORTALITY; PATIENT; FAILURE;
D O I
10.1002/1873-3468.14444
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Mitochondrial dysfunction is known to contribute to a range of diseases, and primary mitochondrial defects strongly impact high-energy organs such as the heart. Platforms for high-throughput and human-relevant assessment of mitochondrial diseases are currently lacking, hindering the development of targeted therapies. In the past decade, human-induced pluripotent stem cells (iPSCs) have become a promising technology for drug discovery in basic and clinical research. In particular, human iPSC-derived cardiomyocytes (iPSC-CMs) offer a unique tool to study a wide range of mitochondrial functions and possess the potential to become a key translational asset for mitochondrial drug development. This review summarizes mitochondrial functions and recent therapeutic discoveries, advancements and limitations of using iPSC-CMs to study mitochondrial diseases of the heart with an emphasis on cardiac applications.
引用
收藏
页码:1735 / 1745
页数:11
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