Entrez Summary: This gene encodes a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. The encoded protein is a mitochondrial enzyme that functions in catabolism of the branched-chain amino acid valine. Defects in this gene are the cause of isobutyryl-CoA dehydrogenase deficiency.[provided by RefSeq, Nov 2009].
UniProt Summary: Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex. {ECO:0000269|PubMed:12359132}.
Pfam DomainsGO Terms
Pfam Domains
Acyl-CoA dh 2
Acyl-CoA dh M
Acyl-CoA dh 1
Acyl-CoA dh N
GO Terms
valine catabolic process
valine metabolic process
acyl-CoA dehydrogenase activity
branched-chain amino acid metabolic process
branched-chain amino acid catabolic process
flavin adenine dinucleotide binding
alpha-amino acid catabolic process
cellular amino acid catabolic process
alpha-amino acid metabolic process
carboxylic acid catabolic process
organic acid catabolic process
cellular amino acid metabolic process
mitochondrial matrix
small molecule catabolic process
carboxylic acid metabolic process
oxidation-reduction process
oxoacid metabolic process
organic acid metabolic process
organonitrogen compound catabolic process
lipid metabolic process
small molecule metabolic process
organic substance catabolic process
cellular catabolic process
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)