Long QT Syndrome modelling with cardiomyocytes derived from human-induced pluripotent stem cells

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2 Citations (Scopus)

Abstract

Long QT syndrome (LQTS) is a potentially severe arrhythmogenic disorder, associated with a prolonged QT interval and sudden death, caused by mutations in key genes regulating cardiac electrophysiology. Current strategies to study LQTS in vitro include heterologous systems or animal models. Despite their value, the overwhelming power of genetic tools has exposed the many limitations of these technologies. In 2010, human-induced pluripotent stem cells (hiPSCs) revolutionised the field and allowed scientists to study in vitro some of the disease traits of LQTS on hiPSC-derived cardiomyocytes (hiPSC-CMs) from LQTS patients. In this concise review we present how the hiPSC technology has been used to model three main forms of LQTS and the severe form of LQTS associated with mutations in calmodulin. We also introduce some of the most recent challenges that must be tackled in the upcoming years to successfully shift hiPSC-CMs from powerful in vitro disease modelling tools into assets to improve risk stratification and clinical decision-making.

Original languageEnglish
Pages (from-to)105-110
Number of pages6
JournalArrhythmia and Electrophysiology Review
Volume8
Issue number2
DOIs
Publication statusPublished - Mar 1 2019

Fingerprint

Long QT Syndrome
Induced Pluripotent Stem Cells
Cardiac Myocytes
Cardiac Electrophysiology
Technology
Mutation
Calmodulin
Sudden Death
Animal Models
Genes
In Vitro Techniques

Keywords

  • Cardiac arrhythmias
  • Cardiomyocytes
  • Human-induced pluripotent stem cells
  • Life-threatening arrhythmias
  • Long QT syndrome
  • Precision medicine
  • Stem cells
  • Sudden cardiac death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

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AU - Gnecchi, Massimiliano

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