Figure 2.

Changes in gene expression of pathways related to sulfide metabolism in response to CoQ₁₀ supplementation. (A and B) Changes in the expression of genes related to cysteine biosynthesis and sulfide metabolism in control (A) and mutant (B) cells after CoQ₁₀ supplementation. (C and D) Changes in the expression of genes related to serine/glycine biosynthesis in control (C) and mutant (D) cells after CoQ₁₀ supplementation. (E and F) Changes in the expression of genes related to the folate cycle in control (E) and mutant (F) cells after CoQ₁₀ supplementation. (G and H) Changes in the expression of genes related to nucleotides metabolism in control (G) and mutant (H) cells after CoQ₁₀ supplementation. Bars in blue indicate genes overexpressed; bars in orange indicates genes underexpressed. Dotted lines indicate the threshold considered for overexpression (log2 FC = 0.585) or underexpression (log2 FC = −0.585). Asterisks indicates the genes that exceed the fold change threshold and have P-values less than 0.05 in the comparison of treated and untreated cells in both control and mutant cells. C, control; CT, control treated with CoQ₁₀; M, NDUFS1 mutant; MT, NDUFS1 mutant treated with CoQ₁₀. (I) Serine/Glycine ratio in control (black bars) and patients’ cells (red bars) with mutations in NDUFS1 and NDUFB3, and their response to CoQ₁₀ supplementation (checkered bars). Data are expressed as mean ± SD. ^**P < 0.01; differences versus control. ⁺P < 0.05; ⁺⁺P < 0.01; control versus control + CoQ₁₀ or Complex I deficiency versus Complex I deficiency + CoQ₁₀ (t-test; n = 3 for each group; n = 3 for each group).