Entrez Summary: This gene encodes the enzyme responsible for hydrolysis of both HIBYL-CoA and beta-hydroxypropionyl-CoA. Mutations in this gene have been associated with 3-hyroxyisobutyryl-CoA hydrolase deficiency. Alternative splicing results in multiple transcript variants.[provided by RefSeq, May 2010].
UniProt Summary: Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA. {ECO:0000269|PubMed:8824301}.
Pfam DomainsGO Terms
Pfam Domains
ECH
GO Terms
3-hydroxyisobutyryl-CoA hydrolase activity
valine catabolic process
valine metabolic process
branched-chain amino acid metabolic process
branched-chain amino acid catabolic process
fatty acid beta-oxidation
fatty acid oxidation
lipid oxidation
fatty acid catabolic process
alpha-amino acid catabolic process
monocarboxylic acid catabolic process
cellular amino acid catabolic process
cellular lipid catabolic process
lipid modification
alpha-amino acid metabolic process
organic acid catabolic process
carboxylic acid catabolic process
lipid catabolic process
cellular amino acid metabolic process
fatty acid metabolic process
mitochondrial matrix
small molecule catabolic process
monocarboxylic acid metabolic process
carboxylic acid metabolic process
cellular lipid metabolic process
oxidation-reduction process
oxoacid metabolic process
organic acid metabolic process
organonitrogen compound catabolic process
lipid metabolic process
mitochondrion
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)