| Description | D-2-Hydroxyglutarate Dehydrogenase (D2HGDH: EC 1.1.99.2) is a mitochondrial enzyme which catalyzes the conversion of D-2-Hydroxyglutarate into 2-Oxoglutarate. In humans, mutation of D2HGDH gene could reduce D2HGDH activity which will cause the D-2-Hydroxyglutarate accumulation in body fluids. Increased D-2-Hydroxyglutarate level in body fluids leads to D-2-Hydroxyglutaric Aciduria, a neurometabolic disorder characterized as developmental delay, epilepsy, hypotonia, and dysmorphic features. BioVision's PicoProbe? D-2-Hydroxyglutarate Dehydrogenase Assay kit provides a quick and easy method for monitoring D2HGDH activity in a wide variety of samples. In this assay, D2HGDH converts D-2-Hydroxyglutarate into 2-Oxoglutarate and NADH, which |
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