glutaryl-CoA dehydrogenase
Symbol
GCDH
(may also be known as: None)
Organism
Human
Description
The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It catalyzes 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. 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. A related pseudogene has been identified on chromosome 12. [provided by RefSeq, Mar 2013]
Orthologs
| Symbol | Species | |
|---|---|---|
| F54D5.7 | Caenorhabditis elegans | |
| CG9547 | Drosophila melanogaster | |
| gcdhl | Danio rerio | |
| Gcdh | Mus musculus | |
| Gcdh | Rattus norvegicus |
Links to external resources
Changes associated with this gene
GO Terms
| GO ID | GO Term | GO Category | |
|---|---|---|---|
| GO:0006554 | lysine catabolic process | biological_process | |
| GO:0006637 | acyl-CoA metabolic process | biological_process | |
| GO:0019395 | fatty acid oxidation | biological_process | |
| GO:0034641 | cellular nitrogen compound metabolic process | biological_process | |
| GO:0044281 | small molecule metabolic process | biological_process | |
| GO:0046949 | fatty-acyl-CoA biosynthetic process | biological_process | |
| GO:0005739 | mitochondrion | cellular_component | |
| GO:0005743 | mitochondrial inner membrane | cellular_component | |
| GO:0005759 | mitochondrial matrix | cellular_component | |
| GO:0000062 | fatty-acyl-CoA binding | molecular_function | |
| GO:0003995 | acyl-CoA dehydrogenase activity | molecular_function | |
| GO:0004361 | glutaryl-CoA dehydrogenase activity | molecular_function | |
| GO:0050660 | flavin adenine dinucleotide binding | molecular_function | |