Susceptibility to Ventricular Arrhythmias Resulting from Mutations in FKBP1B, PXDNL, and SCN9A Evaluated in hiPSC Cardiomyocytes
Representative recordings from a WT (a) and MMRL1126 hiPSC-CM (b). Current recordings were obtained at test potentials between −80 and 25 mV in 5 mV increments. The holding potential was −120 mV. (c) I–V relation for WT () and MMRL1126 () showing no difference in magnitude. (d) Steady-state activation relation for WT and MMRL1126 hiPSC-CM cells. Chord conductance was determined using the ratio of current to the electromotive potential for the cells shown in (a) and (b). Data were normalized and plotted against their test potential. Representative steady-state inactivation recordings from a WT (e) and MMRL1126 hiPSC-CM (f). The voltage clamp protocol is shown at the top of the figure. Peak current was normalized to their respective maximum values and plotted against the conditioning potential. The mean data for the steady-state inactivation relation is shown (g).
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