Figure 3 | Scientific Reports

Figure 3

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

Figure 3

Transmission electron microscopy analysis of the differentiated Megaconial CMD myotubes. The electron micrographs of the control (A, C, E, G) and the patient (B, D, F, H). Note the abundant and pleomorphic, giant mitochondria in the patient’s sample. Mitochondria contain vacuoles and/or electron dense globular inclusions in (D, F, H). Giant, degenerated mitochondria present abnormal cristae and condensed structure in (B, D, F, H). Nucleus appears healthy in (A) and (B). (G) and (H) presents rough endoplasmic reticulum cisternae next to mitochondria. Uranyl acetate, lead citrate. N: Nucleus, n: Nucleolus, m: Mitochondria, i: Inclusion, mp: Mitophagic Vacuoles, rER: Rough Endoplasmic Reticulum. (A) 15,000×, (B) 12,000×, (C, D) 25,000×, (E, F) 40,000×, (G) 20,000×, (H) 25,000×. The graphs in the lower panel present median, min., max., values of the mitochondrial length and width for the patient and control myotubes. **p < 0.01, ***p < 0.001. Measurements were conducted using at least 50 mitochondria in both patient and control myotubes.

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