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For the electron coupling assays, respiratory substrates (10 mM pyruvate + 2 mM malate, 10 mM succinate + 2 M rotenone, 80 M palmitoyl-carnitine or octanoyl-carnitine + 0.5 mM malate or 10 mM glutamate + 10 mM malate) were diluted in MAS buffer with BSA (70 mM sucrose, 220 mM mannitol, 10 mM KH 2 PO 4 , 5 mM MgCl 2 , 2 mM HEPES and 1 mM EGTA, pH 7.2, in 0.2% FA-free BSA) and added to mitochondria

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The MAT1A gene is located on chromosome 10q22.3 and is composed of 9 exons that encode a protein of 395 amino acids

Recently, electrophysiological measurements have provided evidence for ascorbate efflux via an anion channel which is activated by gluconic acid and blocked by anthracene-9-carboxylic acid [117]