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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.
Gene Name: | enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase |
Synonyms: | EHHADH, 3,2-trans-enoyl-CoA isomerase, ECHD, LBFP, L-PBE, Enoyl-coa hydratase, PBE, PBFE |
Target Sequences: | NM_001966 NP_001957.2 Q08426 |
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