Modeling Inherited Cardiac Disorders

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  • Sallam Karim
    Stanford Cardiovascular Institute Department of Medicine, Division of Cardiology Institute of Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine
  • Kodo Kazuki
    Stanford Cardiovascular Institute Department of Medicine, Division of Cardiology Institute of Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine
  • Wu Joseph C.
    Stanford Cardiovascular Institute Department of Medicine, Division of Cardiology Institute of Stem Cell Biology and Regenerative Medicine, Stanford University School of Medicine

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  • Modeling Inherited Cardiac Disorders : A Cell Is Worth a Thousand Genes
  • – A Cell Is Worth a Thousand Genes –

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Abstract

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.  (Circ J 2014; 78: 784–794)<br>

Journal

  • Circulation Journal

    Circulation Journal 78 (4), 784-794, 2014

    The Japanese Circulation Society

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