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Modelling the long QT syndrome with induced pluripotent stem cells

Author

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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
    Rambam Medical Center, the Bruce Rappaport Faculty of Medicine, Technion — Israel Institute of Technology, POB 9649)

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.

Suggested Citation

  • Ilanit Itzhaki & Leonid Maizels & Irit Huber & Limor Zwi-Dantsis & Oren Caspi & Aaron Winterstern & Oren Feldman & Amira Gepstein & Gil Arbel & Haim Hammerman & Monther Boulos & Lior Gepstein, 2011. "Modelling the long QT syndrome with induced pluripotent stem cells," Nature, Nature, vol. 471(7337), pages 225-229, March.
    • Handle: RePEc:nat:nature:v:471:y:2011:i:7337:d:10.1038_nature09747
    DOI: 10.1038/nature09747
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    Cited by:

    1. Sungjin Min & Suran Kim & Woo-Sup Sim & Yi Sun Choi & Hyebin Joo & Jae-Hyun Park & Su-Jin Lee & Hyeok Kim & Mi Jeong Lee & Inhea Jeong & Baofang Cui & Sung-Hyun Jo & Jin-Ju Kim & Seok Beom Hong & Yeon, 2024. "Versatile human cardiac tissues engineered with perfusable heart extracellular microenvironment for biomedical applications," Nature Communications, Nature, vol. 15(1), pages 1-22, December.

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