Figure 3 | Scientific Reports

Figure 3

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

Figure 3

Characterization of hERG trafficking defect in LQTS2-iPSC cardiomyocytes. (a) Immunostaining for sarcomeric α-actinin (red) and hERG (green) in hiPSC-CM. Control cardiomyocytes exhibit membrane and intracellular hERG localization. A higher magnification of the membrane region marked by the dashed rectangle is shown on the right. Observe that, in LQTS2-iPSCs and LQTS2-CRISPR, hERG staining is reduced when compared to control. Quantification of hERG fluorescence intensity signal in the membrane (b) and intracellular space (c). CTRL-iPSC (n = 25); LQTS2-iPSC1 (n = 22); LQTS2-iPSC2 (n = 20); LQTS2-CRISPR (n = 17). Box plots represent 1st quartile, median and 3rd quartile. Whiskers represent minimum and maximum values. **p < 0.01, ****p < 0.0001. Scale bar: 10 µm.

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