【摘 要】
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Aims Inherited long QT syndrome (LQTS) is characterized by QT-interval prolongation and lethal arrhythmia resulting from dysfunctions of cardiac ion channels.Previous studies on LQTS have been hampere
【机 构】
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National Heart Centre Singapore National Heart an
【出 处】
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The 4th International Ion Channel Conference(第四届国际离子通道会议)
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Aims Inherited long QT syndrome (LQTS) is characterized by QT-interval prolongation and lethal arrhythmia resulting from dysfunctions of cardiac ion channels.Previous studies on LQTS have been hampered by the lack of patient-derived cardiomyocytes, the most relevant disease model, due to safety concerns and technical challenges.This study aims to establish a patient-specific human induced pluripotent stem cell (hiPSC) model of LQTS.Methods and Results LQTS patients clinically and genetically confirmed as Type 1 (LQT1, with mutation in KCNQ1), type 2 (LQT2, with mutation in KCNH2) and type 3 (LQT3, with mutation in SCN5A) which collectively account for over 50% of the LQTS patients, were en-rolled.
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