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L-α-Hydroxyglutaric acid disodium salt is a salt form of L-α-Hydroxyglutaric acid. L-α-Hydroxyglutaric acid accumulates as a result of a rare defect in L-2-HG dehydogenase, resulting in the metabolic disorder L-α-hydroxyglutaric aciduria.

In vitro: It has been reported that L-α-Hydroxyglutaric acid disodium salt could be used as the collection buffer for increasing the recovery of hypoxia-inducible factor-1 α, which was a marker of hypoxia in human tumors [1]. In another previous study, L-α-Hydroxyglutaric acid disodium salt was also used in the radiolabeled conversion assay to evaluate its effect on the TET family of methyl hydroxylases [2].

In vivo: In the 30-day-old rats, L-α-Hydroxyglutaric acid was found to be able to significantly increase chemiluminescence, PCF and TBA-RS measurements and decrease the TAR values in cerebellum markedly. In addition, the L-α-Hydroxyglutaric acid-induced increase of TBA-RS was significantly attenuated by melatonin and also by the combinations of ascorbic acid and soluble alpha-tocopherol (Trolox) and of superoxide dismutase plus catalase but not by the inhibitor of nitric oxide synthase Nomega-nitro-L-arginine methyl ester (L-NAME), , superoxide dismutase or creatine or catalase alone in either cerebral structure [3].

Clinical trial: Up to now, L-a-Hydroxyglutaric acid disodium salt is still in the preclinical development stage.

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