Uncovering Inherited Cardiomyopathy With Human Induced Pluripotent Stem Cells.

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 . 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 using patient-specific hiPSC-derived cardiomyocytes. In this review, we will make a summary of recent advances in inherited cardiomyopathy modeling using hiPSCs.