title: Recapitulating long-QT syndrome using induced pluripotent stem cell technology.
abstract: The generation of patient-specific stem cells by reprogramming somatic cells to induced pluripotent stem cells (iPSC) provides the basis for a promising new type of in vitro disease models. Patient-specific iPSC derived from individuals with hereditary disorders can be differentiated into somatic cells in vitro, thus allowing the pathophysiology of the diseases to be studied on a cellular level. Different types of long-QT syndrome have been successfully modeled using this approach, demonstrating that the iPSC-derived patient-specific cardiomyocytes recapitulated key features of the disease in vitro. This approach will likely serve to model other monogenetic or polygenetic cardiovascular disorders in the future. Moreover, test platforms based on patient-specific iPSC could be used to test the potential of drug candidates to induce QT-interval prolongation or other unwanted side effects, screen for novel cardiovascular drugs, or to tailor medical therapy to the specific needs of a single patient.
authors.affiliation: Medizinische Klinik-Kardiologie, Klinikum rechts der Isar-Technische Universität München, Ismaninger Straße 22, 81675, München, Germany.
authors.forename: Ralf J
authors.initials: RJ
authors.lastname: Dirschinger
authors.forename: Alexander
authors.initials: A
authors.lastname: Goedel
authors.forename: Alessandra
authors.initials: A
authors.lastname: Moretti
authors.forename: Karl-Ludwig
authors.initials: KL
authors.lastname: Laugwitz
authors.forename: Daniel
authors.initials: D
authors.lastname: Sinnecker
mesh_terms: D002454:Cell Differentiation; D005347:Fibroblasts / Q000166:cytology*; D006801:Humans; D066298:In Vitro Techniques; D057026:Induced Pluripotent Stem Cells / Q000378:metabolism; D008133:Long QT Syndrome / Q000378:metabolism / Q000503:physiopathology*; D032383:Myocytes, Cardiac / Q000378:metabolism*