Product Description: Price
Αlpha-ketoglutarate (oxoglutarate) dehydrogenase complex (aKGDC), a Krebs Cycle enzyme complex, catalyzes the oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA with concomitant production of NADH. The enzyme complex together with pyruvate dehydrogenase complex (PDC) and branched-chain ketoacid dehydrogenase complex (BCKDC) belong to the alpha-ketoacid dehydrogenase complex family. Each of these enzyme complexes is a multiunit complex consisting of unique E1 (alpha-ketoacid dehydrogenase) and E2 (dihydrolipoyl transacetylase) subunits as well as an identical E3 subunit (dihydrolipoyl dehydrogenase), which catalyzes the transfer of two hydrogen atoms from dihydrolipoic acid to its prosthetic group FAD and restores oxidized lipoic acid. The reduced FADH2 of the E3 subunit then transfer H+ to NAD+ to form NADH. aKGDC represents a major modulator of oxidative phosphorylation and Krebs Cycle flux, and is involved in the metabolic remodeling following a cancer cell’s transformation in bioenergetic requirements. Deficiency of the enzyme complex has been described as a variant form of maple syrup urine disease (MSUD) as a result of deficiency of the E3 component, leading to PDC and BCKDC deficiencies. This non-radioactive colorimetric aKGDC assay is based on the reduction of the tetrazolium salt INT in a NADH-coupled enzymatic reaction to formazan, which is water-soluble and exhibits an absorption maximum at 492 nm. The intensity of the red color formed is increased in the presence of increased aKGDC activity. The kit is stable for many years under proper storage and handling conditions.
#KrebsCycle #alpha-KetoglutarateDehydrogenaseComplex #branchedchainaminoacid
#MSUD #MapleSyrupUrineDisease
Kit Components:
aKGDC Assay Solution: 5 ml (for 100 wells), store at -80ºC
20x Cofactors: 0.25 ml, store at -80ºC
20x KGDC Substrate: 0.25 ml, store at -80ºC
10x Lysis Buffer: 25 ml, store at 4ºC
10x Extraction Buffer: 20 ml, store at 4ºC
SDS:
DMSO, INT, Tris
Related Kits:
PDC Assay, BCKDC Assay