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Modelling the long QT syndrome with induced pluripotent stem cells

Ilanit Itzhaki, Leonid Maizels, Irit Huber, Limor Zwi-Dantsis, Oren Caspi, Aaron Winterstern, Oren Feldman, Amira Gepstein, Gil Arbel, Haim Hammerman, Monther Boulos and Lior Gepstein ()
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Ilanit Itzhaki: Sohnis Family Research Laboratory for Cardiac Electrophysiology and Regenerative Medicine, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Leonid Maizels: Sohnis Family Research Laboratory for Cardiac Electrophysiology and Regenerative Medicine, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Irit Huber: Sohnis Family Research Laboratory for Cardiac Electrophysiology and Regenerative Medicine, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Limor Zwi-Dantsis: Sohnis Family Research Laboratory for Cardiac Electrophysiology and Regenerative Medicine, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Oren Caspi: Sohnis Family Research Laboratory for Cardiac Electrophysiology and Regenerative Medicine, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Aaron Winterstern: Sohnis Family Research Laboratory for Cardiac Electrophysiology and Regenerative Medicine, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Oren Feldman: Sohnis Family Research Laboratory for Cardiac Electrophysiology and Regenerative Medicine, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Amira Gepstein: Sohnis Family Research Laboratory for Cardiac Electrophysiology and Regenerative Medicine, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Gil Arbel: Sohnis Family Research Laboratory for Cardiac Electrophysiology and Regenerative Medicine, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Haim Hammerman: Rambam Medical Center, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Monther Boulos: Rambam Medical Center, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649
Lior Gepstein: Sohnis Family Research Laboratory for Cardiac Electrophysiology and Regenerative Medicine, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649

Nature, 2011, vol. 471, issue 7337, 225-229

Abstract: Stem-cell model for long QT Long QT syndrome (LQTS) is a life-threatening congenital condition characterized by cardiac arrhythmias and sudden cardiac death. A model for LQTS type 2, which is caused by a mutation in the potassium-channel-encoding KCNH2 gene, has now been developed using induced pluripotent stem-cell technology to generate cardiomyocytes from the fibroblasts of an individual diagnosed with the syndrome. The resulting cells were used to screen a variety of drugs for either amelioration or aggravation of the disease phenotype, demonstrating the power of this approach for drug development and for patient-specific safety screening.

Date: 2011
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DOI: 10.1038/nature09747

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