Fig. 5: Morphometric analysis of mitochondria in TARDBP-ALS fibroblasts.

Mitochondrial network in TARDBP-ALS family (A). Active mitochondria are shown by MitoTracker Red (Orange) and the total mitochondrial mass by MitoTracker (green). Digital Phase Contrast (Gray) has been added to show the cells shapes, also contoured in gray. Nuclei are stained in blue (Hoechst). Scale Bars = 10μm. Mitochondrial ratio (B), mass (C) and membrane potential (D). Data represented the mean of three independent experiments and results are shown as the mean ± SEM. Statistical significance: * p < 0.05, **p < 0.01 (One-way ANOVA test with Tukey’s correction). Representative Western blot analysis of OPA1 (80-100 kDa) and MFF (25–35 kDa) (E) and their expression levels in unrelated controls and TARDBP-ALS family fibroblasts compared to loading control (Vinculin 121 kDa) (F, G).