ECHS1

Enoyl Coenzyme A hydratase, short chain, 1, mitochondrial, also known as ECHS1, is a is_associated_with::human is_associated_with::gene.

Structure
The ECHS1 gene is approximately 11 kb in length, and is composed of eight is_associated_with::exons, with exons I and VIII containing the 5'- and 3'-untranslated regions, respectively. There are two major transcription start sites, located 62 and 63 bp upstream of the translation codon, were mapped by primer extension analysis. The 5'-flanking region of the ECHS1 gene is GC-rich and contains several copies of the SP1 binding motive but no typical TATA or CAAT boxes are apparent. Alu repeat elements have been identified within the region -1052/-770 relative to the cap site and in is_associated_with::intron 7. The precursor is_associated_with::polypeptide contains 290 is_associated_with::amino acid residues, with an N-terminal presequence of 29 residues, a 5'-untranslated sequence of 21 bp and a 3'-untranslated sequence of 391 bp.

Function
Enoyl-CoA hydratase (ECH) catalyzes the second step in is_associated_with::beta-oxidation pathway of fatty acid metabolism. The enzyme is involved in the formation of a β-hydroxyacyl-CoA is_associated_with::thioester. The two catalytic glutamic acid residues are believed to act in concert to activate a water molecule, while Gly-141 is proposed to be involved in substrate activation. There are two potent inhibitors of ECHS, which irreversibly inactivate the enzyme via covalent adduct formation.

Clinical significance
Enoyl-CoA hydratase short chain has been confirmed to interact with is_associated_with::STAT3, such that ECHS1 specifically represses STAT3 activity by inhibiting STAT3 phosphorylation. STAT3 can act as both an oncogene and a tumor suppressor. ECHS1 itself has shown to occur in many cancers, particularly in is_associated_with::hypatocellular carcinoma (HCC) development; both exogenous and endogenous forms of ECHS1 bind to HBs and induce apoptosis as a result. This means that ECHS1 may be used in the future as a therapy for patients with HBV-related is_associated_with::hepatitis or HCC.