Purification notesab112998 was produced in vitro using hybridomas grown in serum-free medium, and then purified by biochemical fractionation. Monoclonal purity was near homogeneity as judged by SDS-PAGE.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 1 µg/ml.
Use at an assay dependent concentration.
Use a concentration of 1 µg/ml. 0.1 µg/well.
Use a concentration of 1 µg/ml. 0.1% Triton X-100 permeabilization recommended.
ab170190-Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.
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.
ab112998 at 1 µg/ml staining GCDH in fibroblast cells by immunocytochemistry (4% paraformaldehyde fixed and 0.1% Triton X-100 permeabilized) followed by Alexa Fluor® 594 goat anti-mouse IgG (H+L) used at a 1/1000 dilution for 1 hour (red). Nuclei were stained with DAPI.