Fig. 1

Characterization of iPSC-derived cardiac fibroblasts from DMD and control patients. A: Western blot analysis of dystrophin protein expression in human iPSC-derived cardiac fibroblasts. 80 µg of iPSC-derived cardiac fibroblasts and 10 µg of heart were loaded, transferred on membrane and revealed with monoclonal antibody against the C-terminal end of the protein. Three major bands at 427 kDa, 71 kDa and 40 kDa were observed in control patients whereas DMD patients expressed only the 40 kDa and faint amount of 71 kDa. B: Representative Flow cytometry graphs obtained with iPSC-derived cardiac fibroblasts generated from DMD and control patients. Cells were first identified on a forward scatter/side scatter (FSC-A/SSC-A) dot plot, doublets were removedand live cells were selected. Cells expressed different levels of CD29, CD105, CD90 (thy1.1) and PDGFRα. Endothelial and leucocyte markers, CD31 and CD45 respectively, were negative (not shown). C: Flow cytometry quantification of expressed markers Data are presented as mean ± SEM