1.1.99.2 (S)-2-hydroxyglutarate + acceptor - 1.1.99.2 (S)-2-hydroxyglutarate + acceptor enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria 1.1.99.2 (S)-2-hydroxyglutarate + FAD enzyme deficiency might be the cause of L-2-hydroxyglutaric aciduria 1.1.99.2 (S)-2-hydroxyglutarate + NAD+ - 1.1.99.2 L-2-hydroxyglutarate + FAD - 1.1.99.2 L-2-hydroxyglutarate + NAD+ - 1.1.99.2 additional information high levels of the enzyme is produced at the late stage of cultivation in the presence of citrate and with limited aeration 1.1.99.2 additional information mutations (K81E, E176D, DELTA-exon9) found in patients with L-2-hydroxyglutaric aciduria supress L-2-hydroxyglutarate dehydrogenase activity. L-2-hydroxyglutartic aciduria is due to a deficiency in L-2-hydroxyglutarate dehydrogenase