Key result
Patient-specific iPSC-CMs successfully model electrophysiological and morphological features of hereditary cardiac disorders in vitro.
Why the study?
Advances in understanding and treatment of cardiac disorders have been limited by the inability to study beating human cardiac cells in vitro.
iPSC-derived cardiomyocytes provide a robust in vitro platform for modeling inherited cardiac disorders, including channelopathies and cardiomyopathies.
iPSC-CM models advance preclinical study of hereditary disorders; leaves open validation for clinical translation or drug screening.
Advances in the understanding and treatment of cardiac disorders have been thwarted by the inability to study beating human cardiac cells in vitro. Induced pluripotent stem cells (iPSCs) bypass this hurdle by enabling the creation of patient-specific iPSC-derived cardiomyocytes (iPSC-CMs). These cells provide a unique platform to study cardiac diseases in vitro, especially hereditary cardiac conditions. To date, iPSC-CMs have been used to successfully model arrhythmic disorders, showing excellent recapitulation of cardiac channel function and electrophysiologic features of long QT syndrome types 1, 2, 3, and 8, and catecholaminergic polymorphic ventricular tachycardia (CPVT). Similarly, iPSC-CM models of dilated cardiomyopathy (DCM) and hypertrophic cardiomyopathy (HCM) have shown robust correlation of predicted morphologic, contractile, and electrical phenotypes. In addition, iPSC-CMs have shown some features of the respective phenotypes for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), LEOPARD syndrome, Pompe's disease, and Friedriech's ataxia. In this review, we examine the progress of utilizing iPSC-CMs as a model for cardiac conditions and analyze the potential for the platform in furthering the biology and treatment of cardiac disorders.
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Sallam et al. (2014) conducted a review in Hereditary cardiac disorders. Induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) was evaluated. Patient-specific induced pluripotent stem cell-derived cardiomyocytes successfully model the electrophysiological and morphological features of hereditary cardiac disorders in vitro.
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