title: Modeling long QT syndromes using induced pluripotent stem cells: current progress and future challenges.
abstract: Long QT syndromes (LQTS) are a family of inherited monogenetic disorders caused by gain or loss-of-function mutations of cardiac ion channels and are characterized by a prolonged QT interval in the ECG. The disease-specific mutations lead to prolonged action potential durations and early after-depolarizations in cardiomyocytes potentially giving rise to triggered extrabeats and life-threatening arrhythmias in patients. The generation of induced pluripotent stem cells from somatic cells of patients and their differentiation into cardiomyocytes represents a powerful method enabling the investigation of disease-specific cardiomyocytes. In this review we highlight the latest progress in the generation of long QT syndrome-specific induced pluripotent stem cells and cardiomyocytes to investigate the disease in vitro. We also point out future challenges that need to be addressed to allow drug screening using patient-specific cardiomyocytes.
authors.affiliation: Institute of Physiology I, Life & Brain Center, University of Bonn, Sigmund-Freud Str. 25, Bonn 53105, Germany.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Friedrichs
authors.forename: Daniela
authors.initials: D
authors.lastname: Malan
authors.forename: Philipp
authors.initials: P
authors.lastname: Sasse
mesh_terms: D018929:Cell Culture Techniques / Q000639:trends; D002454:Cell Differentiation / Q000235:genetics; D005544:Forecasting; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000378:metabolism / Q000473:pathology; D007473:Ion Channels / Q000235:genetics; D008133:Long QT Syndrome / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D008955:Models, Cardiovascular; D009154:Mutation; D032383:Myocytes, Cardiac / Q000378:metabolism / Q000473:pathology; D018408:Patch-Clamp Techniques
chemical_list: D007473:Ion Channels