The cell growth of the patient’s fibroblasts is lowered in galactose medium and is partially rescued by CoQ10 supplementation. (A) Fibroblast cultures from two controls and Patient II-2 after 4 days of cell growth, stained for nucleus (DAPI) in blue and actin (Phalloidin) in green. Scale bar = 300 μm. (B) Number of cells per field in the galactose medium after 4 days of growth, normalized to the number of cells in glucose medium, in the fibroblasts of the patient (#AF400) and controls (#AB249 and #V972) (Kruskal–Wallis test followed by Dunn’s multiple comparison test; ***P < 0.001; n = 3). (C) Number of cells per field in the optimal medium during 4 days of growth, normalized to the number of cells at Day 0 in the optimal medium, in the fibroblasts of the patient (#AF400) and controls (#AB249 and #V972). (D) Change in the number of cells per field in galactose medium over 4 days, normalized to the number of cells at Day 0 in glucose medium, in the fibroblasts of the patient (#AF400) and controls (#AB249 and #V972) (two-way ANOVA followed by Bonferroni tests **P < 0.01; n = 3). (E) Number of cells per field in glucose, galactose, and galactose with 10 μM CoQ10 medium after 3 days in culture, normalized to the number of cells at Day 0 in glucose medium, in the fibroblasts of the patient (#AF400) and controls (#V972) (two-way ANOVA followed by Bonferroni tests **P < 0.01; n = 3).