Enoyl-coenzyme A hydratase (ECHS1) is a mitochondrial protein which catalyzes the hydration of 2-trans-enoyl-coenzyme A intermediates to L-3-hydroxyacyl-coenzyme A,playing key role in metabolism of fatty acids in mitochondria. ECHS1 is highly expressed in muscle,liver and fibroblasts. Altered expression of ECHS1 has been considered as a characteristic feature of mitochondria dysfunction. (23275097,23235493)