Product Description: Price

Branched-chain alpha-ketoacid dehydrogenase complex (BCKDC) catalyzes the oxidative decarboxylation of branched-chain alpha-ketoacid (derived from metabolism of leucine, isoleucine, and valine) to branched-chain CoA derivatives with concomitant production of NADH. The enzyme complex together with pyruvate dehydrogenase complex (PDC) and alpha-ketoglutarate dehydrogenase complex (KGDC) 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. The mammalian BCKDC also contains two regulatory enzymes: a specific kinase and phosphatase that regulate BCKDC through phosphorylation (inactivation) and dephosphorylation (activation). Inborn errors in BCKDC cause maple syrup urine disease (MSUD, 1 in ~200,000), which is included in many neonatal screening programs. This non-radioactive colorimetric BCKDC 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 BCKDC activity. The kit is stable for many years if stored and handled properly.

#BCKDC #Branched-chainalpha-KetoacidDehydrogenaseComplex #leucine #isoleucine #valine #MSUD #MapleSyrupUrineDIsease

Kit Components:

BCKDC Assay Solution: 5 ml (100 wells), store at -80ºC

20x Cofactors: 0.25 ml, store at -80ºC

20x BCKDC Substrate: 0.25 ml, store at -80ºC

10x Lysis Buffer: 25 ml, store at 4ºC

10x Extraction Buffer: 20 ml, store at 4ºC

SDS:

TX-100, DMSO, INT, Tris

Related Kits:

PDC, alpha-KGDC