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Glutaryl-CoA dehydrogenase, mitochondrial (GCDH)

UniProt Number: Q92947
Alternate Names: GCD
Structure and Function: Glutaryl-CoA dehydrogenase is an acyl dehydrogenase involved in the metabolism of lysine, hydroxylysine, and tryptophan. Specifically, it catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO2 in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor.
Disease Associations: More than 150 mutations in GCDH are known. Defects 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.

Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount
MS781 GCDH antibody human, rat, mouse ICC, IP, ICE, Flow 100 µg

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