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L-2-hydroxyglutarate dehydrogenase metabolic scheme

In this metabolic scheme illustrating the normal role of L-2-hydroxyglutarate dehydrogenase, L-malate dehydrogenase interconverts the citric acid intermediates oxaloacetate and L-malate using NADH/NAD+. L-malate dehydrogenase also converts alpha-ketoglutarate to L-2-OH-glutarate, but the reaction equilibrium is shifted towards L-2-OH-glutarate. Therefore, another enzyme, L-2-OH-glutarate dehydrogenase, is needed to convert L-2-OH-glutarate back to alpha-ketoglutarate and prevent toxic accumulation of L-2-OH-glutarate. (Adapted from: Rzem R, Veiga-da-Cunha M, Noel G, et al. A gene encoding a putative FAD-dependent L-2-hydroxyglutarate dehydrogenase is mutated in L-2-hydroxyglutaric aciduria. Proc Natl Acad Sci U S A 2004;101(48):16849-54.)

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