Protein Name enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase
Organism Homo sapiens
Gene ID 1962
Gene Symbol

EHHADH

UniProt Q08426 (ECHP_HUMAN)
Relationships Total Number of functionally related compound(s) : 92
Total Number of Articles : 66
Description

enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase

Gene Summary

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. [provided by RefSeq, Oct 2009]

synonyms
  • peroxisomal bifunctional enzyme
  • 3,2-trans-enoyl-CoA isomerase
  • L-3-hydroxyacyl-CoA dehydrogenase
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Properties