Figure 4

Western Blot and quantification analysis of proteins regulating mitochondrial dynamics in primary skeletal muscle cells of the Megaconial CMD patient. (a) Western blot images of proteins involved in mitochondrial fusion (MFN1: 84 kDa, MFN2: 86 kDa, OPA1: 80-100 kDa) and fission (DRP1: 79-84 kDa), MFF: 25-38 kDa), FIS1: 15 kDa). While actin was used to control equal loading, muscle specific expression patterns of proteins were eveluated by comparing the levels of proteins to desmin. (b) Graphs showing the quantitative analysis of Western blotting by ImageJ. The asterisk in the graph indicates the significant p value obtained by Student's t-test (**p < 0.01, ***p < 0.001), and the error bar indicates the standard deviation. Uncropped images of Western blotting are shown in Supplementary figure S1.