InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: GCDH

Summary

InnateDB Protein

IDBP-31279.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

GCDH

Protein Name

glutaryl-CoA dehydrogenase

Synonyms

ACAD5; GCD;

Species

Homo sapiens

Ensembl Protein

ENSP00000222214

InnateDB Gene

IDBG-31277 (GCDH)

Protein Structure

UniProt Annotation

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. {ECO:0000269PubMed:17176108, ECO:0000269PubMed:6423663, ECO:0000269PubMed:8541831}.

Subcellular Localization

Mitochondrion matrix.

Disease Associations

Glutaric aciduria 1 (GA1) [MIM:231670]: An autosomal recessive metabolic disorder characterized by progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia. {ECO:0000269PubMed:14707522, ECO:0000269PubMed:8541831, ECO:0000269PubMed:8900227, ECO:0000269PubMed:8900228, ECO:0000269PubMed:9600243}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Isoform 1 and isoform 2 are expressed in fibroblasts and liver.

Comments

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

Protein Structure and Domains

PDB ID

InterPro

IPR006091 Acyl-CoA oxidase/dehydrogenase, central domain
IPR009075 Acyl-CoA dehydrogenase/oxidase C-terminal
IPR009100 Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain
IPR013107 Acyl-CoA dehydrogenase, C-terminal domain
IPR013786 Acyl-CoA dehydrogenase/oxidase, N-terminal

PFAM

PF02770
PF00441
PF08028
PF02771

PRINTS

PIRSF

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q92947

PhosphoSite