PicoProbe D-2-Hydroxyglutarate Dehydrogenase Assay Kit (Fluorometric)

Catalog number: K248-100
Brand: BioVision
Packing: 100 assays
Price: € 588.00
Expected delivery time: 14 days
Quantity:

Product specifications for - PicoProbe D-2-Hydroxyglutarate Dehydrogenase Assay Kit (Fluorometric)

Overview: 
Product group: Assays
Category: Activity
Application: Detection of D-2-Hydroxyglutarate Dehydrogenase activity
Species: Mammals
Properties: 
Assay Sample Type: Purified recombinant protein. Cell and tissue lysate
Assay Test Principle: Fluorescence (Ex/Em=535/587 nm)
Datasheet: Datasheet
  Research Use Only
UNSPSC: 41116133
Form supplied: D2HGDH Assay Buffer. PicoProbe (in DMSO). D2HGDH Substrate. D2HGDH Developer. D2HGDH Positive Control. NADH Standard
Scientific information: 
Scientific info: 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 uU/ul of D2HGDH activity.