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Table 2 Action potential parameters of ventricular-like human induced pluripotent stem cell cardiomyocytes treated with ML277

From: 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

Spontaneous APs

APA (mV)

APD50 (ms)

APD90 (ms)

APD90/APD50

Overshoot (mV)

MDP (mV)

Heart rate (beats/minute)

LQT1 patient

Baseline (n = 11)

98.4 ± 1.7

601.1 ± 51.1

665.4 ± 51.9

1.12 ± 0.01

40.1 ± 2.4

−60.9 ± 1.6

64.6 ± 6.0

ML277 (1 μM) (n = 11)

98.1 ± 1.3

455.5 ± 44.6**

514.7 ± 46.0**

1.15 ± 0.02

38.0 ± 2.8

−61.1 ± 1.7

66.9 ± 1.6

Control (father)

Baseline (n = 9)

99.1 ± 3.5

315.1 ± 23.1

368.8 ± 22.5

1.13 ± 0.01

41.5 ± 1.1

−59.4 ± 1.1

75.8 ± 6.8

ML277 (1 μM) (n = 9)

99.2 ± 2.4

228.0 ± 24.4**

288.4 ± 27.4**

1.17 ± 0.02

40.7 ± 2.7

−60.6 ± 1.5

76.0 ± 8.0

  1. 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 <0.01, versus baseline.