The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. Defects in this gene are a cause of peroxisomal disorders such as Zellweger syndrome. Two transcript variants encoding different isoforms have been found for this gene.
EHHADH; enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase; ECHD, enoyl Coenzyme A, hydratase/3 hydroxyacyl Coenzyme A dehydrogenase; peroxisomal bifunctional enzyme; 3 hydroxyacyl CoA dehydrogenase; 3,2 trans enoyl CoA isomerase; 3-hydroxyacyl-CoA dehydrogenase; ECHD; ECHP_HUMAN; Enoyl Coenzyme A, hydratase/3 hydroxyacyl Coenzyme A dehydrogenase; L 3 hydroxyacyl CoA dehydrogenase; L bifunctional protein, peroxisomal; L PBE; LBFP; LBP; MGC120586; MS730; PBE; PBFE; Peroxisomal enoyl CoA hydratase; 3,2-trans-enoyl-CoA isomerase; peroxisomal enoyl-CoA hydratase; L-3-hydroxyacyl-CoA dehydrogenase; L-bifunctional protein, peroxisomal; enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase; L-PBE; LBP, ECHD, LBFP, PBFE, L-PBE; zgc:77526; DKEYP-30D5.2