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Figure 1

From: Reduced mitochondrial fission and impaired energy metabolism in human primary skeletal muscle cells of Megaconial Congenital Muscular Dystrophy

Figure 1

Mitochondrial morphology and network analysis in primary skeletal muscle cells of the Megaconial CMD patient (a) Immunofluorescence staining of the differentiated myotubes of the Megaconial CMD patient and the control by anti-TOM20 antibody (red), with nuclei counterstained with DAPI (blue). Original images were processed and skeletonized by ImageJ 1.53c software (NIH, https://imagej.nih.gov/ij/), as indicated. (b) Graphs showing the results of mitochondrial morphometric analysis. For all comparisons, differences between data sets were assessed by Mann–Whitney U-test with **p < 0.01, ***p < 0.001. (c) Images of mitochondrial morphology and network structure in myoblasts, myotubes and fibroblasts of the Megaconial CMD patient. Green/FITC: desmin/myoblasts and myotubes; Red/Texas Red: TOM20/mitochondria; blue: DAPI/nuclei. Scale bars: 10 μm. Analysis was performed on 50 primary skeletal muscle cells in both patient and control.

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