The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application notesWB: 1/500 - 1/1000. Detects bands of approximately 46 and 48 kDa (predicted molecular weight: 48 kDa).
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
FunctionCatalyzes 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.
Tissue specificityIsoform 1 and isoform 2 are expressed in fibroblasts and liver.
Involvement in diseaseDefects in GCDH are the cause of glutaric aciduria type 1 (GA1) [MIM:231670]. GA1 is an autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.
Sequence similaritiesBelongs to the acyl-CoA dehydrogenase family.