Modeling long-QT syndromes with iPS cells

Daniel Sinnecker, Alexander Goedel, Tatjana Dorn, Ralf J. Dirschinger, Alessandra Moretti, Karl Ludwig Laugwitz

Research output: Contribution to journalArticlepeer-review

18 Scopus citations

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.

Original languageEnglish
Pages (from-to)31-36
Number of pages6
JournalJournal of Cardiovascular Translational Research
Volume6
Issue number1
DOIs
StatePublished - Feb 2013
Externally publishedYes

Keywords

  • Disease modeling
  • LQT
  • Long-QT syndrome
  • Reprogramming
  • iPS cells

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