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

Biology

Razan Elfadil Ahmed¹  Hideki Uosaki¹  Tatsuya Anzai¹  Nawin Chanthra¹  Takeshi Tokuyama¹ 

[1] Division of Regenerative Medicine, Center for Molecular Medicine, Jichi Medical University, Shimotsuke 329-0498, Japan;

关键词: mitochondrial disease;    mitochondrial cardiomyopathy;    induced pluripotent stem cells (iPSC);    iPSC-derived cardiomyocyte;   

DOI  :  10.3390/biology10100981

来源: DOAJ

【 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.

【 授权许可】

Unknown