Glutaryl-CoA Dehydrogenase Mitochondrial/GCDH Protein(N-6His) (recombinant human)

Protein Construction: Recombinant Human Glutaryl-CoA Dehydrogenase, Mitochondrial is produced by our E.coli expression system and the target gene encoding Arg45-Lys438 is expressed with a 6His tag at the N-terminus. Sequence: Arg45-Lys438. Fusion tag: N-6His Endotoxin: < 1.0 EU per µg as determined by the LAL method. Apparent Molecular Mass: 41 kDa. Protein function: Glutaryl-CoA Dehydrogenase Mitochondrial (GCDH) is an enzyme that acts upon glutaryl-coenzyme A, creating crotonyl-coenzyme A. It plays a role in the metabolism of lysine, hydroxylysine and tryptophan. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive Glutaryl-CoA and electron-transfer flavoprotein to (E)-but-2-enoyl-CoA, CO2 and reduced electron-transfer flavoprotein. A defect in this enzyme is associated with neurological condition glutaric acidemia type 1 and cause a progressive form of early-onset generalized dystonia. Protein function: 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. [The UniProt Consortium]