Why the study?
Human heart tissues are difficult to obtain and culture in vitro, and animal models differ molecularly from human diseases, making hiPSCs valuable for investigating inherited cardiomyopathies.
Human induced pluripotent stem cells provide a valuable in vitro model for investigating the pathogenic mechanisms of inherited cardiomyopathies, overcoming limitations of animal models and primary human tissue.
hiPSC models advance inherited cardiomyopathy research; extends prior tools but leaves open clinical translation pending validation.
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.
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Jiang et al. (2021) studied this question.
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