Entity Details

Primary name GCDH_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ92947
EntryNameGCDH_HUMAN
FullNameGlutaryl-CoA dehydrogenase, mitochondrial
TaxID9606
Evidenceevidence at protein level
Length438
SequenceStatuscomplete
DateCreated1997-11-01
DateModified2021-06-02

Ontological Relatives

GenesGCDH

GO terms

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GOName
GO:0000062 fatty-acyl-CoA binding
GO:0004361 glutaryl-CoA dehydrogenase activity
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
GO:0006554 lysine catabolic process
GO:0006568 tryptophan metabolic process
GO:0033539 fatty acid beta-oxidation using acyl-CoA dehydrogenase
GO:0046949 fatty-acyl-CoA biosynthetic process
GO:0050660 flavin adenine dinucleotide binding

Subcellular Location

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Subcellular Location
Mitochondrion matrix

Domains

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DomainNameCategoryType
IPR006089 Acyl-CoA dehydrogenase, conserved siteSiteConserved site
IPR006091 Acyl-CoA oxidase/dehydrogenase, central domainDomainDomain
IPR009075 Acyl-CoA dehydrogenase/oxidase C-terminalDomainDomain
IPR009100 Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain superfamilyFamilyHomologous superfamily
IPR013786 Acyl-CoA dehydrogenase/oxidase, N-terminalDomainDomain
IPR036250 Acyl-CoA dehydrogenase-like, C-terminalFamilyHomologous superfamily
IPR037069 Acyl-CoA dehydrogenase/oxidase, N-terminal domain superfamilyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
231670 OMIMGlutaric aciduria 1 (GA1)An autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB03147 Flavin adenine dinucleotideDrugbanksmall molecule
DB03245 S-4-Nitrobutyryl-CoADrugbanksmall molecule