Characterization of a novel KCNQ1 mutation for type 1 long QT syndrome and assessment of the therapeutic potential of a novel IKs activator using patient-specific induced pluripotent stem cell-derived cardiomyocytes

Table 1 Action potential parameters of ventricular-like human induced pluripotent stem cell cardiomyocytes

AP	APA (mV)	APD50 (ms)	APD90 (ms)	APD90/APD50	Overshoot (mV)	MDP (mV)	Heart rate (beats/minute)
Spontaneous
LQT1 patient (n = 41)	98.2 ± 1.3	603.9 ± 39.2***	671.0 ± 41.1***	1.12 ± 0.01	40.8 ± 1.0	−58.7 ± 1.1	69.4 ± 4.7
Control (n = 17)	99.1 ± 2.6	319.3 ± 13.8	372.9 ± 14.2	1.18 ± 0.02	40.0 ± 1.4	−59.8 ± 0.7	71.0 ± 5.2
Paced (1 Hz)
LQT1 patient (n = 30)	100.4 ± 1.1	477.1 ± 23.9***	545.3 ± 26.6***	1.15 ± 0.02	42.2 ± 1.0	−58.3 ± 1.3	60
Control (n = 8)	101.5 ± 6.5	349.9 ± 23.7	397.5 ± 32.2	1.13 ± 0.02	42.4 ± 2.5	−58.3 ± 3.2	60

AP, action potential; APA, action potential amplitude; APD50, action potential duration at 50% repolarization; APD90, action potential duration at 90% repolarization; LQT1, type 1 long QT syndrome; MDP, maximum diastolic potential. ***P <0.005, versus control.

ISSN: 1757-6512