EHHADH Proteins and Enzymes

The protein encoded by the EHHADH gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomalbeta-oxidation pathway. The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while theC-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. Defects in this gene are a cause of peroxisomaldisorders such as Zellweger syndrome. Two transcript variants encoding different isoforms have been found for thisgene. (provided by RefSeq)