Recombinant Human Enoyl-CoA hydratase, mitochondrial (ECHS1)

Uniprot Id

P30084

Target Species

Human

Target Name

ECHS1

Target Full Name

Enoyl-CoA hydratase, mitochondrial

Target Function

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.

Target Involvement

Mitochondrial short-chain enoyl-CoA hydratase 1 deficiency (ECHS1D)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

Enoyl-CoA hydratase/isomerase family

Target Tissue Specificity

Liver, fibroblast, muscle. Barely detectable in spleen and kidney.

Target Research Area

Cancer

Target Synonyms

ECHS1Enoyl-CoA hydratase; mitochondrial; EC 4.2.1.17; Enoyl-CoA hydratase 1; Short-chain enoyl-CoA hydratase; SCEH

Target Background

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.