Homo sapiens Gene: GCDH
Summary
InnateDB Gene IDBG-31277.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol GCDH
Gene Name glutaryl-CoA dehydrogenase
Synonyms ACAD5; GCD
Species Homo sapiens
Ensembl Gene ENSG00000105607
Encoded Proteins
glutaryl-CoA dehydrogenase
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary 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]
Gene Information
Type Protein coding
Genomic Location Chromosome 19:12891026-12914207
Strand Forward strand
Band p13.13
Transcripts
ENST00000222214 ENSP00000222214
ENST00000421816
ENST00000591470 ENSP00000466845
ENST00000588242
ENST00000590627
ENST00000590445 ENSP00000468125
ENST00000585420
ENST00000589039 ENSP00000465618
ENST00000590472 ENSP00000468625
ENST00000585760
ENST00000590530 ENSP00000468452
ENST00000587072 ENSP00000468584
ENST00000591050 ENSP00000467735
ENST00000587832
ENST00000591043
ENST00000588905 ENSP00000465770
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 16 experimentally validated interaction(s) in this database.
Experimentally validated
Total 16 [view]
Protein-Protein 16 [view]
Protein-DNA 0
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Gene Ontology

Molecular Function
Accession GO Term
GO:0000062 fatty-acyl-CoA binding
GO:0003995 acyl-CoA dehydrogenase activity
GO:0004361 glutaryl-CoA dehydrogenase activity
GO:0016627 oxidoreductase activity, acting on the CH-CH group of donors
GO:0050660 flavin adenine dinucleotide binding
Biological Process
GO:0006554 lysine catabolic process
GO:0006568 tryptophan metabolic process
GO:0006637 acyl-CoA metabolic process
GO:0008152 metabolic process
GO:0019395 fatty acid oxidation
GO:0034641 cellular nitrogen compound metabolic process
GO:0044281 small molecule metabolic process
GO:0046949 fatty-acyl-CoA biosynthetic process
GO:0055114 oxidation-reduction process
Cellular Component
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0005759 mitochondrial matrix
Orthologs
Species
Mus musculus
Bos taurus
Gene ID
Gene Order
Not yet available
Pathways
NETPATH
REACTOME
Lysine catabolism pathway
Metabolism of amino acids and derivatives pathway
Metabolism pathway
KEGG
Tryptophan metabolism pathway
Fatty acid degradation pathway
Lysine degradation pathway
INOH
Tryptophan degradation pathway
Lysine degradation pathway
PID NCI
Cross-References
SwissProt
TrEMBL
UniProt Splice Variant
Entrez Gene
UniGene Hs.532699 Hs.666526
RefSeq NM_000159 NM_013976 XM_006722721
HUGO
OMIM
CCDS CCDS12286
HPRD 01977
IMGT
EMBL
GenPept
RNA Seq Atlas