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D-α-Hydroxyglutaric acid, an α-hydroxy acid formed from the hydrolysis of (R)-5-oxo-2-tetrahydrofurancarboxylic acid, is over-produced in the human neurometabolic disease D-α-hydroxyglutaric aciduria.

In vitro: In humans the enzyme hydroxyacid-oxoacid transhydrogenase catalyzed the formation of D-α-hydroxyglutaric acid, while D-α-hydroxyglutaric acid is catalyzed by 2-hydroxyglutarate synthase in bacteria. Recent study discovered heterozygous somatic mutations in the genes encoding isocitrate dehydrogenase-1 and -2 (IDH1 and IDH2) and these mutations could disable the enzymes' ability to convert 2-ketoglutarate to D-α-hydroxyglutarate. The authors also found heterozygous germline mutations in IDH2 that alter enzyme residue in patients with D-2-hydroxyglutaric aciduria, a neurometabolic disorder characterized by supraphysiological levels of D-α-Hydroxyglutaric acid [1].

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