Enoyl Coenzyme A Hydratase Short Chain 1, Mitochondrial (ECHS1)

Enoyl Coenzyme A hydratase, short chain, 1, mitochondrial 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.The predicted 290-amino acid human protein is 87% identical to that of the rat gene. By Northern blot analysis, Kanazawa et al. (1993) found a single 1.6-kb mRNA species transcribed most strongly in human liver, but also in fibroblast and muscle.