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Other Disorders of the Intermediary Metabolism D-2-Hydroxyglutaric aciduria (D-2-HGA) and L-2-hydroxyglutaric aciduria (L-2-HGA) are neurometabolic disorders biochemically characterized by the accumulation of D-2-hydroxyglutaric acid (D-2-HG) and L-2-hydroxyglutaric acid (L-2-HG), respectively, predominantly in the brain and biological fluids of the affected patients [110]