Glutaryl-CoA dehydrogenase
Protein-coding gene in the species Homo sapiens / From Wikipedia, the free encyclopedia
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Glutaryl-CoA dehydrogenase (GCDH) is an enzyme encoded by the GCDH gene on chromosome 19. The protein belongs to the acyl-CoA dehydrogenase family (ACD). It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and carbon dioxide 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.[5]
GCDH | |||||||||||||||||||||||||||||||||||||||||||||||||||
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Identifiers | |||||||||||||||||||||||||||||||||||||||||||||||||||
Aliases | GCDH, ACAD5, GCD, glutaryl-CoA dehydrogenase, Glutaryl-Coenzyme A dehydrogenase | ||||||||||||||||||||||||||||||||||||||||||||||||||
External IDs | OMIM: 608801 MGI: 104541 HomoloGene: 130 GeneCards: GCDH | ||||||||||||||||||||||||||||||||||||||||||||||||||
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glutaryl-CoA dehydrogenase (decarboxylating) | |||||||||
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Identifiers | |||||||||
EC no. | 1.3.8.6 | ||||||||
CAS no. | 37255-38-2 | ||||||||
Databases | |||||||||
IntEnz | IntEnz view | ||||||||
BRENDA | BRENDA entry | ||||||||
ExPASy | NiceZyme view | ||||||||
KEGG | KEGG entry | ||||||||
MetaCyc | metabolic pathway | ||||||||
PRIAM | profile | ||||||||
PDB structures | RCSB PDB PDBe PDBsum | ||||||||
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