AffiASSAY® D-2-Hydroxyglutarate Dehydrogenase Assay Kit (Fluorometric)

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 further reduces PicoProbe™ to generate an intense fluorescent product (Ex/Em = 535/587 nm). This kit is simple, sensitive and high-throughput adaptable and can detect as low as 4 µU/µl of D2HGDH activity.