As an L-hydroxy-specific enzyme, EHHADH (enoyl-CoA-hydratase:3-hydroxyacyl-CoA dehydrogenase), also known as Peroxisomal L-bifunctional enzyme, is a 723 amino acid protein has an essential tripeptide sequence on its carboxyl-terminus that is required for peroxisomal transport. EHHADH-null mice only exhibit a blunted peroxisome proliferative response when challenged with a peroxisome proliferator. Since there were no observed changes in lipid metabolism, this evidence suggests that enoyl-CoAs were diverted to the D-hydroxy-specific beta-oxidation system for metabolism.
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Protein Aliases: 3,2-trans-enoyl-CoA isomerase; 3-hydroxyacyl-CoA dehydrogenase; Enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase; enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase; L-3-hydroxyacyl-CoA dehydrogenase; L-bifunctional protein, peroxisomal; PBE; Peroxisomal bifunctional enzyme; peroxisomal enoyl-CoA hydratase
Gene Aliases: ECHD; EHHADH; FRTS3; L-PBE; LBFP; LBP; PBFE
UniProt ID: (Human) Q08426
Entrez Gene ID: (Human) 1962