Fig. 6

iPSC-derived cardiac fibroblasts from DMD patients displayed abnormal microfilament morphology and mitochondrial network. A: Representative 63 × confocal images of hiPSC-fibroblasts stained with anti-HSP 60 antibodyto show the mitochondrial network in iPSC-derived cardiac fibroblasts generated from control and DMD patients. The dashed white lines show plasma membrane of the cells. Quantification of mitochondrial network using MiNA(Mitochondrial Network Analysis) workflow within Fiji, for the ‘’mitochondrial footprint’’ or mitochondrial dispersion area (left) and the mitochondrial “networks’’(right). A t-test was applied to determine the statistically significant differences between DMD and control. ****p < 0.0001. B: Representative 63 × confocal images of phalloidin-stained filamentous actin and subsequent quantification. C: Western blot and quantification of beta actin, gamma actin, b tubulin and Rho GDI protein expression in iPSC-derived cardiac fibroblasts. Data are presented as mean ± SEM from analyses obtained from different batches of cardiac fibroblasts differentiated from iPSC.Non parametric t-test was applied to determine the statistically significant differences between DMD and control. ***p < 0.001. D: Representative 63 × confocal images of hiPSC-fibroblasts stained with anti β-tubulin antibody showing the microtubule network in iPSC-derived cardiac fibroblasts generated from control and DMD patients