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SCEH / ECHS1 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.
Gene Name: | enoyl CoA hydratase, short chain, 1, mitochondrial |
Synonyms: | ECHS1, Enoyl-CoA hydratase 1, SCEH |
Target Sequences: | NM_004092 NP_004083.3 P30084 |
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