Modeling long-QT syndromes with iPS cells

J Cardiovasc Transl Res. 2013 Feb;6(1):31-6. doi: 10.1007/s12265-012-9416-1. Epub 2012 Oct 18.

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Anti-Arrhythmia Agents / pharmacology
  • Cells, Cultured
  • Genetic Predisposition to Disease
  • Humans
  • Induced Pluripotent Stem Cells / drug effects
  • Induced Pluripotent Stem Cells / metabolism*
  • Ion Channels / drug effects
  • Ion Channels / genetics
  • Ion Channels / metabolism*
  • Long QT Syndrome / chemically induced
  • Long QT Syndrome / drug therapy
  • Long QT Syndrome / genetics
  • Long QT Syndrome / metabolism*
  • Long QT Syndrome / physiopathology
  • Myocytes, Cardiac / drug effects
  • Myocytes, Cardiac / metabolism*
  • Phenotype
  • Risk Assessment
  • Toxicity Tests

Substances

  • Anti-Arrhythmia Agents
  • Ion Channels