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Details on Person UniProt:Q92947 GCDH
| Class:Id | ReferenceGeneProduct:55484 |
|---|---|
| _chainChangeLog | transit peptide:1-44 added on Sat February 7 2015;chain:45-438 added on Sat February 7 2015 |
| _displayName | UniProt:Q92947 GCDH |
| _timestamp | 2025-02-21 19:48:14 |
| chain | transit peptide:1-44 chain:45-438 |
| checksum | 415B8D510027BB63 |
| comment | FUNCTION 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. Isoform Short is inactive.CATALYTIC ACTIVITY glutaryl-CoA + oxidized [electron-transfer flavoprotein] + 2 H(+) = (2E)-butenoyl-CoA + reduced [electron-transfer flavoprotein] + CO2COFACTOR Strongly inhibited by MCPA-CoA, a metabolite of hypoglycin which is present in unripened fruit of the ackee tree.BIOPHYSICOCHEMICAL PROPERTIES Release of crotonyl-CoA product from the enzyme is the rate-determining step in its steady-state turnover.PATHWAY Amino-acid metabolism; lysine degradation.PATHWAY Amino-acid metabolism; tryptophan metabolism.SUBUNIT Homotetramer.SUBCELLULAR LOCATION Isoform Long and isoform Short are expressed in fibroblasts and liver.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the acyl-CoA dehydrogenase family. |
| description | recommendedName: Glutaryl-CoA dehydrogenase, mitochondrial shortName: GCD ecNumber: 1.3.8.6 |
| geneName | GCDH |
| identifier | Q92947 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation Alternative splicing Disease variant FAD Flavoprotein Glutaricaciduria Mitochondrion Oxidoreductase Proteomics identification Reference proteome Transit peptide |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 |
| name | GCDH |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8999502] ENSEMBL:ENSG00000105607 GCDH [Homo sapiens] |
| secondaryIdentifier | GCDH_HUMAN A8K2Z2 O14719 |
| sequenceLength | 438 |
| species | [Species:48887] Homo sapiens |
| (isoformParent) | [ReferenceIsoform:147829] UniProt:Q92947-2 GCDH [Homo sapiens] [ReferenceIsoform:412522] UniProt:Q92947-1 GCDH [Homo sapiens] |
| (referenceEntity) | [EntityWithAccessionedSequence:71039] GCDH [mitochondrial matrix] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:Q92947 GCDH (55484)
