Entrez Summary: The protein encoded by this gene functions in the second step of the mitochondrial fatty acid beta-oxidation pathway. It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) intermediates to L-3-hydroxyacyl-CoAs. The gene product is a member of the hydratase/isomerase superfamily. It localizes to the mitochondrial matrix. Transcript variants utilizing alternative transcription initiation sites have been described in the literature. [provided by RefSeq, Jul 2008].
UniProt Summary: Straight-chain enoyl-CoA thioesters from C4 up to at least C16 are processed, although with decreasing catalytic rate. Has high substrate specificity for crotonyl-CoA and moderate specificity for acryloyl-CoA, 3-methylcrotonyl-CoA and methacrylyl-CoA. It is noteworthy that binds tiglyl-CoA, but hydrates only a small amount of this substrate. {ECO:0000269|PubMed:26251176}.
Pfam DomainsGO Terms
Pfam Domains
ECH
GO Terms
enoyl-CoA hydratase activity
fatty acid beta-oxidation
fatty acid oxidation
lipid oxidation
fatty acid catabolic process
monocarboxylic acid catabolic process
cellular lipid catabolic process
lipid modification
carboxylic acid catabolic process
organic acid catabolic process
lipid catabolic 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
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)