Fig. 5

NF1 Stabilizes NADH-linked OXPHOS and mitochondrial electron flow in Schwann cells. (A) Schematic overview of oxidative phosphorylation and electron transfer pathways. (B-C) Oxidation of pyruvate and malate in the LEAK state. (D-E) Oxidative phosphorylation and electron transfer in the presence of pyruvate (P), malate (M), and glutamate (G). (F-G) Oxidative phosphorylation and electron transfer in the presence of succinate (S). (H-I) Oxidative phosphorylation and electron transfer in the presence of pyruvate, malate, glutamate, and succinate. (J) Rate of electron transfer in presence of glycerol 3-phosphate(G3P). (K) Complex IV (CIV) activity supported by TMPD and ascorbate. NF1 +/+ and -/- cells were compared using an unpaired t-test. Data are displayed as a box (mean ± 5–95% confidence interval) and whiskers (minimum to maximum) with all data points (colored dots). (LEAK (L): Respiratory state with saturating O2 concentration and substrate, and low to no adenylate charge. OXPHOS (P): Respiratory state with saturating O2 concentration, substrate, and saturating ADP (ATP: ADP ratio ≈ 0). ET (E): Respiratory state with saturating O2 concentration, substrate, and noncoupled mitochondria (ΔG’ATP ≈ 0). PM = pyruvate and malate).