Mitochondrial oxidation of TCA cycle intermediates, fatty acids, amino acids and other substrates in lymphoblasts from ALS patients with mutant SOD1 (mutSOD1) or unknown mutation (undSOD1) and respective controls.

Cells were plated at density 160,000 cells/well, and the rate of oxidation of a panel of TCA cycle intermediates, fatty acids, amino acids and other substrates was assessed using Biolog Mitoplate S1 assay. Data were obtained for all cohorts (pool), C1-Female 46 years old, C2- Male 46 years old and C3-Male 26/27 years old. Substrates include pyruvic acid, pyruvic acid + malic acid, citric acid, cis-aconitic acid, isocitric acid, α-ketoglutaric acid, succinic acid, fumaric acid, L-malic acid, L-glutamic acid, L-glutamine and alanine-glutamine, octanoyl L-carnitine (+L-malic acid), palmitoyl L-carnitine (+L-malic acid), D-glucose, D-glucose-1-PO4, D-glucose-6-PO4, D-gluconate-6-PO4, L-lactic acid, D-L-α-glycerol-PO4, glycogen, D-L-β-hydroxybutyric acid, α-ketobutyric acid, γ-aminobutyric acid (+L-Malic acid), tryptamine, serine, L-ornithine, and L-leucine (+L-malic acid).