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. 2013 Jan;141(1):61–72. doi: 10.1085/jgp.201210899

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© 2013 Terrenoire et al.

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Figure 7. — Mexiletine corrects F1473C-altered Na⁺ channel inactivation. (A and B, top) Averaged I_Na current traces recorded at −10 mV (100-ms pulses applied at 0.2 Hz from a −90-mV holding potential) in father (A; WT, n = 5, clone HR-I-2R 2Cr) and proband (B; LQT-3, n = 18, clone OA6 9Cr8) iPSC-CMs in control conditions (gray traces and arrows) and in the presence of 50 µM mexiletine (black traces and arrows) at low and high (insets) gain. (insets) I_Na at high gain shows the absence of mexiletine-sensitive I_NaL in father’s iPSC-CMs (A) but reveals the presence of I_NaL in LQT-3 iPSC-CMs, which was significantly blocked by 50 µM mexiletine (B, arrow). (A and B, bottom) Steady-state availability in the absence (open symbols) and presence (closed symbols) of 50 µM mexiletine in WT (A; n = 5, clone HR-I-2R 2Cr) and LQT-3 (B; n = 8, clone OA6 9Cr8) cells. Data are shown as means ± SEM.