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Induced pluripotent stem cell-derived cardiomyocytes in studies of inherited arrhythmias

Journal

JOURNAL OF CLINICAL INVESTIGATION
Volume 123, Issue 1, Pages 84-91

Publisher

AMER SOC CLINICAL INVESTIGATION INC
DOI: 10.1172/JCI62838

Keywords

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Funding

  1. Telethon [GGP11141]
  2. CARIPLO [pr.2008.2275]
  3. Fondation Leducq [08CVD01]
  4. Fondazione Veronesi Award on inherited arrhythmogenic diseases
  5. Ministero Salute [GR2010_2315996]

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The discovery of the genetic basis of inherited arrhythmias has paved the way for an improved understanding of arrhythmogenesis in a wide spectrum of life-threatening conditions. In vitro expression of mutations and transgenic animal models have been instrumental in enhancing this understanding, but the applicability of results to the human heart remains unknown. The ability to differentiate induced pluripotent stem cells (iPSs) into cardiomyocytes enables the potential to generate patient-specific myocytes, which could be used to recapitulate the features of inherited arrhythmias in the context of the patient's genetic background. Few studies have been reported on iPS-derived myocytes obtained from patients with heritable arrhythmias, but they have demonstrated the applicability of this innovative approach to the study of inherited arrhythmias. Here we review the results achieved by iPS investigations in arrhythmogenic syndromes and discuss the existing challenges to be addressed before the use of iPS-derived myocytes can become a part of personalized management of inherited arrhythmias.

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