Function
Enoyl-CoA hydratase (ECH) (gene rpfF) is part of the fatty acid metabolism (Beta oxidation). ECH hydrates the double bond between the second and third carbons in acyl-CoA. ECH1 or short-chain ECH is mitochondrial. ECH1 processes enoyl-CoA thioesters from C4 to C16[1]. Enoyl-CoA hydratase/isomerase catalyzes the hydration and hydrolysis of methylthioacryloyl-CoA in the last step of the methanethiol pathway of dimethyl-sulphoniopropionate catabolism[2].
Disease
ECH1 mutations cause combined respiratory chain deficiency resulting inn Leigh syndrome[3].
Structural highlights
; . ECH . [4]
3D structures of enoyl-CoA hydratase
Enoyl-CoA hydratase 3D structures