IUBMB Enzyme Nomenclature


Accepted name: phytanoyl-CoA dioxygenase

Reaction: phytanoyl-CoA + 2-oxoglutarate + O2 = 2-hydroxyphytanoyl-CoA + succinate + CO2

Glossary entries
phytanate: 3,7,11,15-tetramethylhexadecanoate

Other name(s): phytanoyl-CoA hydroxylase

Systematic name: phytanoyl-CoA, 2-oxoglutarate:oxygen oxidoreductase (2-hydroxylating)

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

Links to other databases: BRENDA, EXPASY, KEGG, Metacyc, PDB, CAS registry number: 185402-46-4


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 (1998) 203-211. [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 (1996) 205-210. [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 (1997) 190-193. [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 (1995) 545-551.

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 (1997) 185-189. [PMID: 9326939]

[EC created 2000]

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