Fig. 3.

NADH production increases with age in control but not in ALS fibroblasts in the presence of fructose energy substrates.

(A) NADH production in fibroblasts in the presence of fructose (B) NADH production in fibroblasts in presence of D-turanose. Controls (N=15) shown in black and ALS (SALS and FALS n=21) group showed in pink. Data presented as mean with standard deviation of at least three biological repeats per cell line. Pearson’s correlation analysis was performed with the p value set to ≤ 0.05. (C) NADH metabolism in the presence of fructose in iAstrocytes. (D) NADH metabolism in the presence of turanose in iAstrocytes. Two-way annova with Sidaks’s post-test analysis was performed on three control lines (black) and eight ALS lines (pink) performed in triplicate. Data presented as mean with standard error. (E) NADH production in fructose with age in iAstrocytes. (F) NADH production in turanose with age in iAstrocytes. Data presented as mean with standard deviation. (G) The effect of fructose supplementation on iAstrocyte mitochondrial function. (H) The effect of fructose supplementation on iAstrocyte glycolytic function. (I) The effect of pyruvate supplementation on iAstrocyte mitochondrial function. (J) The effect of pyruvate supplementation on iAstrocyte glycolytic function. (G-J) Data presented as mean with standard deviation of two control and two ALS cases performed in triplicate. Data was analysed using unpaired t-test analysis with a Welch correction. MR = mitochondrial respiration. CR = coupled respiration. SRC = spare respiratory capacity. GF = glycolytic flux. GC = glycolytic capacity. GR = glycolytic reserve. *p ≤ 0.05, **p ≤ 0.01. (Color version of figure is available online)