Product nameD2HGDH Assay Kit (Fluorometric)
Sample typeCell culture supernatant, Tissue, Adherent cells, Suspension cells
D2HGDH Assay Kit (Fluorometric) (ab239706) 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 the probe 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.
Storage instructionsStore at -20°C. Please refer to protocols.
Components 100 tests D2HGDH Assay Buffer 1 x 20ml D2HGDH Developer 1 vial D2HGDH Positive Control 1 vial D2HGDH Substrate 1 x 0.4ml NADH Standard 1 vial Probe in DMSO 1 x 0.2ml
RelevanceCatalyzes the oxidation of D-2-hydroxyglutarate to alpha-ketoglutarate. Disease: Defects in D2HGDH are the cause of D-2-hydroxyglutaric aciduria type 1 (D2HGA1) [MIM:600721]. D2HGA1 is a rare recessive neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. Both a mild and a severe phenotype exist. The severe phenotype is homogeneous and is characterized by early infantile-onset epileptic encephalopathy and cardiomyopathy. The mild phenotype has a more variable clinical presentation. Diagnosis is based on the presence of an excess of D-2-hydroxyglutaric acid in the urine. Similarity: Belongs to the FAD-binding oxidoreductase/transferase type 4 family. Contains 1 FAD-binding PCMH-type domain.
- D-2-hydroxyglutarate dehydrogenase
- D-2-hydroxyglutarate dehydrogenase, mitochondrial
ab239706 has not yet been referenced specifically in any publications.