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Details on Person UniProt:Q92947 GCDH

Class:IdReferenceGeneProduct:55484
_chainChangeLogtransit peptide:1-44 added on Sat February 7 2015;chain:45-438 added on Sat February 7 2015
_displayNameUniProt:Q92947 GCDH
_timestamp2025-02-21 19:48:14
chaintransit peptide:1-44
chain:45-438
checksum415B8D510027BB63
commentFUNCTION 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.
descriptionrecommendedName: Glutaryl-CoA dehydrogenase, mitochondrial shortName: GCD ecNumber: 1.3.8.6
geneNameGCDH
identifierQ92947
isSequenceChangedFALSE
keyword3D-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
nameGCDH
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8999502] ENSEMBL:ENSG00000105607 GCDH [Homo sapiens]
secondaryIdentifierGCDH_HUMAN
A8K2Z2
O14719
sequenceLength438
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)