Fig. 3
BrS iPSC-CMs carrying SCN5A T1788fs exhibit Nav1.5 defects and arrhythmic phenotypes. A Bar graph to compare the mRNA expression of SCN5A between control (CON) and BrS iPSC-CMs by qPCR. n = 7–8 technical replicates. ****p < 0.0001. B Western blot analysis of Nav1.5 expression in control and BrS iPSC-CMs. Full-length blots are presented in Additional file 1: Fig. S5. C Bar graph to compare the Nav1.5 expression between control and BrS iPSC-CMs. n = 3–4 culture replicates. ****p < 0.0001. D Representative sodium current tracings isolated from control and BrS iPSC-CMs. E Comparison of IV curve of sodium current between control and BrS iPSC-CMs. F Bar graph to compare peak sodium current density at − 20 mV between control and BrS iPSC-CMs. n = 8 cells. ****p < 0.0001. G Comparison of steady-state activation (SSA) and steady-state inactivation (SSI) of sodium current between control and BrS iPSC-CMs. H, I Bar graphs to compare V1/2 of SSA and SSI of sodium current between control and BrS iPSC-CMs. **p < 0.01. J Representative action potential tracings recorded by single-cell patch clamp from control and BrS ventricular-like myocytes. Dashed lines indicate 0 mV. Red arrows indicate putative arrhythmias in BrS iPSC-CMs. K Bar graph to compare the percentage of iPSC-CMs with arrhythmias between control and BrS iPSC-CMs. n = 12–29 cells