Treatment of patients with long-QT syndrome: Differentiation of patient-derived induced pluripotent stem cells into functional cardiac myocytes
Sinnecker D, Dorn T, Dirschinger RJ, Goedel A, Moretti A, Laugwitz KL (2013)
Publication Type: Authored book
Publication year: 2013
Publisher: Springer Netherlands
ISBN: 9789400756458
DOI: 10.1007/978-94-007-5645-8_9
Abstract
The long-QT syndrome, which can be inherited (e.g. due to mutations in genes encoding cardiac ion channels) or acquired (e.g. due to the application of certain drugs), is characterized by a prolonged QT interval in the ECG which is caused by prolonged action potentials of single cardiomyocytes. Affected patients are susceptible to ventricular arrhythmias like torsades de pointes, which lead to palpitations, syncopes, or even sudden cardiac death. Induced pluripotent stem cell lines generated from skin fibroblasts of patients affected by congenital long-QT syndromes can be -differentiated to cardiomyocytes, in which the pathophysiology of the disease can be studied in vitro. These patient-specific cardiomyocytes recapitulate typical features of the disease like a prolonged action potential duration, a defect of the physiological shortening of the action potential duration under conditions of increased heart rate, and a susceptibility to arrhythmogenic early afterdepolarizations. Moreover, the therapeutic effect of beta receptor antagonists can be recapitulated in vitro in these cells. Such patient-specific induced pluripotent stem cell models of the long-QT syndrome might be used in the future to screen for new drugs, to avoid unwanted drug side effects, and to individualize drug therapy.
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Germany (DE)How to cite
APA:
Sinnecker, D., Dorn, T., Dirschinger, R.J., Goedel, A., Moretti, A., & Laugwitz, K.L. (2013). Treatment of patients with long-QT syndrome: Differentiation of patient-derived induced pluripotent stem cells into functional cardiac myocytes. Springer Netherlands.
MLA:
Sinnecker, Daniel, et al. Treatment of patients with long-QT syndrome: Differentiation of patient-derived induced pluripotent stem cells into functional cardiac myocytes. Springer Netherlands, 2013.
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