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

FEBS Letters

article

Arianne Caudal¹  Lu Ren¹  Chengyi Tu¹  Joseph C. Wu¹ 

[1] Stanford Cardiovascular Institute, Stanford University School of Medicine;Institute for Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine;Division of Cardiology, Department of Medicine, Stanford University School of Medicine

关键词: cardiomyocyte;    cardiovascular disease;    heart;    iPSC;    mitochondria;    stem cell;   

DOI  :  10.1002/1873-3468.14444

来源: John Wiley & Sons Ltd.

【 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.

【 授权许可】

Unknown