EC - Phytanoyl-CoA dioxygenase

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IntEnz Enzyme Nomenclature


Accepted name:
phytanoyl-CoA dioxygenase
Other names:
phytanoyl-CoA hydroxylase
phytanoyl-CoA alpha-hydroxylase
phytanoyl-CoA 2-hydroxylase
phytanoyl-CoA 2 oxoglutarate dioxygenase
Systematic name:
phytanoyl-CoA, 2-oxoglutarate:oxygen oxidoreductase (2-hydroxylating)




Part of the peroxisomal phytanic acid α-oxidation pathway. Requires Fe2+ and ascorbate.

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Structural data: CSA , EC2PDB
Gene Ontology: GO:0048244


  1. Jansen, G.A., Mihalik, S.J., Watkins, P.A., Jakobs, C., Moser, H.W. and Wanders, R.J.A.
    Characterization of phytanoyl-CoA hydroxylase in human liver and activity measurements in patients with peroxisomal disorders.
    Clin. Chim. Acta 271: 203-211 (1998). [PMID: 9565335]
  2. Jansen, G.A., Mihalik, S.J., Watkins, P.A., Moser, H.W., Jakobs, C., Denis, S. and Wanders, R.J.A.
    Phytanoyl-CoA hydroxylase is present in human liver, located in peroxisomes, and deficient in Zellweger syndrome: direct, unequivocal evidence for the new, revised pathway of phytanic acid α-oxidation in humans.
    Biochem. Biophys. Res. Commun. 229: 205-210 (1996). [PMID: 8954107]
  3. Jansen, G.A., Ofman, R., Ferdinandusse, S., Ijlst, L., Muijsers, A.O., Skjeldal, O.H., Stokke, O., Jakobs, C., Besley, G.T.N., Wraith, J.E. and Wanders, R.J.A.
    Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene.
    Nat. Genet. 17: 190-193 (1997). [PMID: 9326940]
  4. Mihalik, S.J., Rainville, A.M. and Watkins, P.A.
    Phytanic acid α-oxidation in rat liver peroxisomes. Production of α-hydroxyphytanoyl-CoA and formate is enhanced by dioxygenase cofactors.
    Eur. J. Biochem. 232: 545-551 (1995).
  5. Mihalik, S.J., Morrell, J.C., Kim, D., Sacksteder, K.A., Watkins, P.A. and Gould, S.J.
    Identification of PAHX, a Refsum disease gene.
    Nat. Genet. 17: 185-189 (1997). [PMID: 9326939]

[EC created 2000]