Abstract
In the past decades, researchers discovered the contribution of genetic defects to the pathogenesis of primary cardiomyopathy and tried to explain the pathogenesis of these diseases by establishing a variety of disease models. Although human heart tissues and primary cardiomyocytes have advantages in modeling human heart diseases, they are difficult to obtain and culture in vitro. Defects developed in genetically modified animal models are notably different from human diseases at the molecular level. The advent of human induced pluripotent stem cells (hiPSCs) provides an unprecedented opportunity to further investigate the pathogenic mechanisms of inherited cardiomyopathies in vitro using patient-specific hiPSC-derived cardiomyocytes. In this review, we will make a summary of recent advances in in vitro inherited cardiomyopathy modeling using hiPSCs.
Highlights
Inherited cardiomyopathy is a kind of myocardial disease with a range of different genetic disorders and epigenetic variations (Fatkin and Graham, 2002)
Two major strategies have been developed for human induced pluripotent stem cells (hiPSCs)-based inherited cardiomyopathy modeling: (1) reprogramming the somatic cells from genetic disease patients into hiPSCs and differentiating them into cardiomyocytes and (2) performing gene editing to introduce pathogenic mutations into hiPSCs of healthy donor and differentiating them into cardiomyocytes
Arrhythmogenic right ventricular is an inherited primary heart muscle disorder characterized by fibrofatty infiltration of the myocardium and cardiomyocyte loss basically in the right ventricle (RV) that may lead to sudden cardiac death (Chen et al, 2020; Jahng et al, 2021)
Summary
Xue Jiang1†, Yihuan Chen1†, Xiaofeng Liu2†, Lingqun Ye1, Miao Yu1, Zhenya Shen, Wei Lei1* and Shijun Hu1*. Stem Cell Research, a section of the journal Frontiers in Cell and Developmental. Researchers discovered the contribution of genetic defects to the pathogenesis of primary cardiomyopathy and tried to explain the pathogenesis of these diseases by establishing a variety of disease models. Human heart tissues and primary cardiomyocytes have advantages in modeling human heart diseases, they are difficult to obtain and culture in vitro. Defects developed in genetically modified animal models are notably different from human diseases at the molecular level. The advent of human induced pluripotent stem cells (hiPSCs) provides an unprecedented opportunity to further investigate the pathogenic mechanisms of inherited cardiomyopathies in vitro using patient-specific hiPSC-derived cardiomyocytes. We will make a summary of recent advances in in vitro inherited cardiomyopathy modeling using hiPSCs
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