Recombinant Human GCDH/GCD protein is a Human Full Length protein, in the 45 to 438 aa range, expressed in Escherichia coli, with >90% purity and suitable for SDS-PAGE, MS.
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Application SDS-PAGE | Reactivity Reacts | Dilution info - | Notes - |
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Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.
GCD, GCDH
Recombinant Human GCDH/GCD protein is a Human Full Length protein, in the 45 to 438 aa range, expressed in Escherichia coli, with >90% purity and suitable for SDS-PAGE, MS.
pH: 8
Constituents: 20% Glycerol (glycerin, glycerine), 1.16% Sodium chloride, 0.316% Tris HCl, 0.077% (R*,R*)-1,4-Dimercaptobutan-2,3-diol
ab98118 is purified by using conventional chromatography techniques.
Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.
Belongs to the acyl-CoA dehydrogenase family.
This product was previously labelled as GCDH
GCDH also known as glutaryl-CoA dehydrogenase is an enzyme involved in the catabolic breakdown of specific amino acids. This protein harbors a mass of approximately 45 kDa and functions primarily within the mitochondria. GCDH expression occurs in various tissues including the liver kidney and brain. The enzyme plays a mechanical role by catalyzing the decarboxylation of glutaryl-CoA to crotonyl-CoA facilitating the removal of carbon dioxide in this biochemical process.
This enzyme acts as an important component in the lysine hydroxylysine and tryptophan degradation pathways. GCDH does not form part of a larger enzyme complex but performs its function independently ensuring the proper degradation and metabolism of amino acids. Its activity aids in maintaining metabolic homeostasis preventing the accumulation of harmful intermediates within cells. GCDH may interact with cofactors like flavin adenine dinucleotide (FAD) to carry out its enzymatic activity.
GCDH plays a significant role in the lysine and tryptophan metabolism pathways. Within these pathways GCDH helps convert glutaryl-CoA a molecule derived from amino acid catabolism into intermediates ultimately leading to energy production. The enzyme's functionality connects with other mitochondrial enzymes like isovaleryl-CoA dehydrogenase (IVD) which also participates in amino acid breakdown. Proper interaction and coordination within these pathways are essential for maintaining metabolic balance in the cell.
GCDH is closely linked to glutaric aciduria type 1 (GA1) an inborn error of metabolism. This disorder arises from mutations in the GCDH gene leading to insufficient enzyme activity and subsequent buildup of glutaric acid. This accumulation can cause severe neurological damage over time if untreated. GA1 can show genetic connections with other metabolic disorders involving enzymes like medium-chain acyl-CoA dehydrogenase (MCAD). Understanding these interactions helps in diagnosing and managing such metabolic disorders.
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SDS-PAGE analysis of ab98118 (3µg) under reducing condition and visualized by coomassie blue stain.
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