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.
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Protein Aliases: enoyl CoA hydratase, short chain, 1, mitochondrial; enoyl Coenzyme A hydratase, short chain 1; enoyl Coenzyme A hydratase, short chain, 1, mitochondrial; Enoyl-CoA hydratase 1; Enoyl-CoA hydratase short chain 1 mitochondrial; Enoyl-CoA hydratase, mitochondrial; Enoyl-CoA hydratase, short chain 1, mitochondrial; enoyl-CoA hydratase, short chain, 1, mitochondrial; mitochondrial short-chain enoyl-coenzyme A hydratase 1; SCEH; short chain enoyl-CoA hydratase; Short-chain enoyl-CoA hydratase
Gene Aliases: C80529; ECHS1; ECHS1D; SCEH