Fig. 7

iPSC-derived cardiac fibroblasts from DMD patients display a myofibroblast phenotype and an increased sensitivity to stress. A: Representative immunoblot of α-sma expression obtained in iPSC-derived cardiac fibroblasts generated from DMD and control patients and quantification. Data are presented as mean ± SEM of the six biological samples. Replicates were performed independently on different differentiation batches. B: Immunofluorescence staining of collagen I in iPSC-derived cardiac fibroblasts generated from DMD and control patients and quantification of α-SMA, COL1A and TNC expression in iPSC derived cardiac fibroblast treated with 10 ng/ml TGFβ (C) or 100 ng/ml angiotensin II (D) for 24 h. One-way ANOVA was applied to determine statistically significant differences between the basal condition and TGFβ or angiotensin treatments