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. 2013 Mar 6;35(16):1078–1087. doi: 10.1093/eurheartj/eht067

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© The Author 2013. Published by Oxford University Press on behalf of the European Society of Cardiology.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc/3.0/), which permits non-commercial use, distribution, and reproduction in any medium, provided that the original authorship is properly and fully attributed; the Journal, Learned Society and Oxford University Press are attributed as the original place of publication with correct citation details given; if an article is subsequently reproduced or disseminated not in its entirety but only in part or as a derivative work this must be clearly indicated. For commercial re-use, please contact journals.permissions@oup.com

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Long-QT syndrome-type 2 hiPSC cardiomyocytes can recapitulate the disease phenotype in vitro. (A) Long-QT syndrome-type 2 cardiomyocytes showed characteristic cardiac muscle striations with cardiac troponin-I staining, and (B) formed ventricular (n = 11), atrial (n = 14), and pacemaker-like (n = 9) myocytes. (C) Long-QT syndrome-type 2 cardiomyocytes had prolonged field potential duration (n = 10) and (D) action potential duration compared with controls, which included cardiomyocytes derived from human embryonic stem cells, the patient's father (long-QT syndrome-type 2-PAT) and mother (long-QT syndrome-type 2-MAT). (E) E4031 (1 µM) treatment caused prolongation in action potential duration of control cardiomyocytes (n = 6), depicting the presence of functional hERG channels, and caused arrhythmias only in long-QT syndrome-type 2 cardiomyocytes (n = 6).