Names & Taxonomy
- Uniprot ID:
- Q92947
- Entry Name:
- GCDH_HUMAN
- Status:
- reviewed
- Protein Names:
- Glutaryl-CoA dehydrogenase, mitochondrial (GCD) (EC 1.3.8.6)
- Gene Names:
- GCDH
- Gene Names Primary:
- GCDH
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 438
- Sequence:
- MALRGVSVRLLSRGPGLHVLRTWVSSAAQTEKGGRTQSQLAKSSRPEFDWQDPLVLEEQLTTDEILIRDTFRTYCQERLMPRILLANRNEVFHREIISEMGELGVLGPTIKGYGCAGVSSVAYGLLARELERVDSGYRSAMSVQSSLVMHPIYAYGSEEQRQKYLPQLAKGELLGCFGLTEPNSGSDPSSMETRAHYNSSNKSYTLNGTKTWITNSPMADLFVVWARCEDGCIRGFLLEKGMRGLSAPRIQGKFSLRASATGMIIMDGVEVPEENVLPGASSLGGPFGCLNNARYGIAWGVLGASEFCLHTARQYALDRMQFGVPLARNQLIQKKLADMLTEITLGLHACLQLGRLKDQDKAAPEMVSLLKRNNCGKALDIARQARDMLGGNGISDEYHVIRHAMNLEAVNTYEGTHDIHALILGRAITGIQAFTASK
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix.
Function
- 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.
- Pathway:
- Amino-acid metabolism; lysine degradation.; Amino-acid metabolism; tryptophan metabolism.
- Catalytic Activity:
- Glutaryl-CoA + electron-transfer flavoprotein = crotonyl-CoA + CO(2) + reduced electron-transfer flavoprotein.
- Cofactor:
- COFACTOR: Name=FAD; Xref=ChEBI:CHEBI:57692;
- Kinetics:
- BIOPHYSICOCHEMICAL PROPERTIES: ; Kinetic parameters: KM=4.7 uM for glutaryl-CoA (at pH 6.5)
- Enzyme Regulation:
- ENZYME REGULATION: Strongly inhibited by MCPA-CoA, a metabolite of hypoglycin which is present in unripened fruit of the ackee tree.
- Active Site:
- ACT_SITE 414 414 Proton acceptor.
- Cross Reference Drug Bank:
- DB03147
- Gene Ontology Go:
- mitochondrial matrix
mitochondrion
electron carrier activity
fatty-acyl-CoA binding
flavin adenine dinucleotide binding
glutaryl-CoA dehydrogenase activity
oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor
cellular nitrogen compound metabolic process
fatty acid beta-oxidation using acyl-CoA dehydrogenase
fatty-acyl-CoA biosynthetic process
lipid homeostasis
lysine catabolic process
small molecule metabolic process
tryptophan metabolic process - Gene Ontology Biological Process:
- cellular nitrogen compound metabolic process
fatty acid beta-oxidation using acyl-CoA dehydrogenase
fatty-acyl-CoA biosynthetic process
lipid homeostasis
lysine catabolic process
small molecule metabolic process
tryptophan metabolic process - Gene Ontology Molecular Function:
- electron carrier activity
fatty-acyl-CoA binding
flavin adenine dinucleotide binding
glutaryl-CoA dehydrogenase activity
oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor - Gene Ontology Cellular Component:
- mitochondrial matrix
mitochondrion - Keywords:
- 3D-structure
Acetylation
Alternative splicing
Complete proteome
Disease mutation
FAD
Flavoprotein
Glutaricaciduria
Mitochondrion
Oxidoreductase
Polymorphism
Reference proteome
Transit peptide - Interacts With:
- P62993; P29474; P49768