Figure 1 | Scientific Reports

Figure 1

From: R534C mutation in hERG causes a trafficking defect in iPSC-derived cardiomyocytes from patients with type 2 long QT syndrome

Figure 1

Differentiation and electrophysiology of iPSC-derived cardiomyocytes. (a) Schematic diagram demonstrating the main steps of the differentiation procedure. (b) Representative action potential recordings of spontaneously contracting ventricular-like cardiomyocytes. Note the red line that marks the end of phase 3 for CTRL-iPSC and the green line that marks the end of phase 3 for LQTS2-iPSC1 and LQTS2-iPSC2. (c,d) Our analysis demonstrates that action potential duration of LQTS2-iPSC1, 2 and CRISPR was significantly longer than that of CTRL-iPSC, as was the triangulation of action potentials (e), implying a longer duration of phase 3. CTRL-iPSC (n = 116); LQTS2-iPSC1 (n = 59); LQTS2-iPSC2 (n = 77); LQTS2-CRISPR (n = 20) from 8 independent differentiations for each cell line. Box plots represent 1st quartile, median and 3rd quartile. Whiskers represent minimum and maximum values. “+” represents the mean for each cell line. (f) Treatment with E4031 caused APD prolongation only in CTRL-iPSC, indicating that hERG does not contribute to repolarization in LQTS2- and CRISPR-iPSC (n = 4). Bars represent mean and SEM. *p < 0.05, ****p < 0.0001.

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