The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It 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. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on chromosome 12. [provided by RefSeq, Mar 2013]
Alternative Names
GCD ACAD5
Molecular Weight(MW)
48 kDa
Cellular Localization
Mitochondrion.
Application
WB
Western blot analysis of extracts from HepG2 cells using GCDH Rabbit mAb at 1:1000.
Application Notes
WB:1:1000-1:5000
Additional Information
Form
Liquid
Storage Instructions
Store at -20°C.Stable for 12 months from date of receipt.
Storage Buffer
Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.