Modeling Long-QT Syndromes with iPS Cells

  • Daniel Sinnecker
  • Alexander Goedel
  • Tatjana Dorn
  • Ralf J. Dirschinger
  • Alessandra Moretti
  • Karl-Ludwig Laugwitz
Article

Abstract

The generation of induced pluripotent stem cells (iPSC) from human somatic cells bears the possibility to generate patient-specific stem cell lines which can serve as a theoretically unlimited source of somatic cells carrying the genotype of the patients. Different types of the long-QT syndrome have been studied by analyzing the phenotype of cardiomyocytes generated from patient-specific iPSC lines. Major aspects of the pathophysiology of long-QT syndrome, like prolonged action potentials, arrhythmia, and the effects of pro- and antiarrhythmic drugs could be recapitulated in these cells. In the future, patient-specific iPSC-derived cardiomyocytes might be used to screen for new drugs, to avoid unwanted drug side effects, and to deepen our understanding on the pathophysiology of long-QT syndromes.

Keywords

LQT Long-QT syndrome Disease modeling iPS cells Reprogramming 

Notes

Acknowledgments

This work was supported by grants from the European Research Council (ERC 261053-CHD-iPS) and the German Research Foundation (La 1238/3-1/4-1, Si 1747/1-1).

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Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Daniel Sinnecker
    • 1
  • Alexander Goedel
    • 1
  • Tatjana Dorn
    • 1
  • Ralf J. Dirschinger
    • 1
  • Alessandra Moretti
    • 1
  • Karl-Ludwig Laugwitz
    • 1
  1. 1.I. Medizinische Klinik Kardiologie, Klinikum rechts der IsarTechnische Universität MünchenMunichGermany

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