Enoyl-CoA hydratase, mitochondrial (ECHS1)

Enoyl-CoA hydratase, mitochondrial (ECHS1), is an enzyme located in the mitochondrial matrix that catalyzes the second step in the beta-oxidation pathway of fatty acid metabolism. Specifically, it hydrates 2-trans-enoyl-CoA intermediates to L-3-hydroxyacyl-CoA thioesters, facilitating the continued degradation of fatty acids for energy production[1][5]. ECHS1 acts primarily on short- and medium-chain fatty acyl-CoA esters (C4–C16)[5]. Deficiency in ECHS1 is associated with severe mitochondrial disorders, especially a form of Leigh Syndrome, due to defective oxidative phosphorylation (OXPHOS) and impaired tricarboxylic acid cycle and mitochondrial bioenergetics[2]. ECHS1 also interacts with key signaling proteins such as STAT3 and has been implicated in cancer biology and apoptosis, particularly in liver diseases like hepatocellular carcinoma[1][2]. The enzyme belongs to the hydratase/isomerase superfamily and is essential for normal energy homeostasis in humans.

Other names

enoyl-CoA hydratase short chain 1short-chain enoyl-CoA hydratase 1Enoyl coenzyme A hydratase, short chain, 1, mitochondrial

Irreversible inhibition via covalent adduct formation with ECHS1 (experimental inhibitors).