Description
Product Usage Information
Western Blotting: 1:1000
Immunohistochemistry (Paraffin): 1:100 - 1:400
Immunofluorescence (Immunocytochemistry): 1:50 - 1:200
Storage
Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.
Protocol
Available protocols: Western Blotting, Immunohistochemistry (Paraffin), Immunofluorescence (Immunocytochemistry)
Specificity / Sensitivity
GCDH (F2P2M) Rabbit Monoclonal Antibody recognizes endogenous levels of total GCDH protein.
Species Reactivity: Human
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Trp50 of human GCDH protein.
Background
Glutaryl-CoA dehydrogenase (GCDH) is a mitochondria matrix enzyme that plays a role in the degradative pathway of lysine, hydroxylysine, and tryptophan by converting glutaryl-CoA to crotonyl-CoA (reviewed in 1). Crotonyl-CoA is further metabolized to acetyl-CoA, which enters the tricarboxylic acid (TCA) cycle for energy production. Mutation of the gene is the cause of glutamic aciduria type I (GAI), an autosomal recessive neurological disorder (2). GCDH deficiency leads to an accumulation of glutaryl-CoA and neurotoxic metabolites including glutaric acid and 3-hydroxyglutaric acid (3). Several studies have demonstrated the importance of GCDH in metabolic reprogramming in cancer. Melanoma cells can develop a dependency on GCDH (4). Mechanistically, loss of GCDH results in increased glutarylation of NRF2, a master transcriptional regulator of stress response genes associated with oxidative and metabolic stress responses, which results in increased NRF2 stability and activity and promotes cell death. Glioblastoma stem cells reprogram lysine catabolism, including upregulation of GCDH, which leads to an increase in histone crotonylation regulating tumor immunity (5). In contrast, in hepatocellular carcinoma (HCC), GCDH suppresses tumor growth and metastasis (6).
Alternate Names
ACAD5; GCD; GCDH; glutaryl-CoA dehydrogenase; Glutaryl-CoA dehydrogenase, mitochondrial; glutaryl-Coenzyme A dehydrogenase; truncated GCDH; truncated glutaryl-CoA dehydrogenase
