Phytanoyl-CoA dioxygenase

From Wikipedia, the free encyclopedia

In enzymology, a phytanoyl-CoA dioxygenase (EC 1.14.11.18) is an enzyme that catalyzes the chemical reaction

phytanoyl-CoA + 2-oxoglutarate + O₂ $\rightleftharpoons$ 2-hydroxyphytanoyl-CoA + succinate + CO₂

The 3 substrates of this enzyme are phytanoyl-CoA, 2-oxoglutarate, and O₂, whereas its 3 products are 2-hydroxyphytanoyl-CoA, succinate, and CO₂.

This enzyme belongs to the family of oxidoreductases, specifically those acting on paired donors, with O2 as oxidant and incorporation or reduction of oxygen. The oxygen incorporated need not be derived from O2 with 2-oxoglutarate as one donor, and incorporation of one atom o oxygen into each donor. The systematic name of this enzyme class is phytanoyl-CoA, 2-oxoglutarate:oxygen oxidoreductase (2-hydroxylating). This enzyme is also called phytanoyl-CoA hydroxylase.

1 Structural studies
2 References
3 External links
- 3.1 Gene Ontology (GO) codes

[edit] Structural studies

As of late 2007, only one structure has been solved for this class of enzymes, with the PDB accession code 2A1X.

[edit] References

IUBMB entry for 1.14.11.18
BRENDA references for 1.14.11.18 (Recommended.)
PubMed references for 1.14.11.18
PubMed Central references for 1.14.11.18
Google Scholar references for 1.14.11.18
Jansen GA, Mihalik SJ, Watkins PA, Jakobs C, Moser HW, Wanders RJ (1998). "Characterization of phytanoyl-Coenzyme A hydroxylase in human liver and activity measurements in patients with peroxisomal disorders". Clin. Chim. Acta. 271: 203–11. PMID 9565335.
Wanders RJ (1996). "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 alpha-oxidation in humans". Biochem. Biophys. Res. Commun. 229: 205–10. PMID 8954107.
OH, Stokke O, Jakobs C, Besley GT, Wraith JE, Wanders RJ (1997). "Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene". Nat. Genet. 17: 190–3. PMID 9326940.
Mihalik SJ, Rainville AM, Watkins PA (1995). "Phytanic acid alpha-oxidation in rat liver peroxisomes. Production of alpha-hydroxyphytanoyl-CoA and formate is enhanced by dioxygenase cofactors". Eur. J. Biochem. 232: 545–51. PMID 7556205.
Mihalik SJ, Morrell JC, Kim D, Sacksteder KA, Watkins PA, Gould SJ (1997). "Identification of PAHX, a Refsum disease gene". Nat. Genet. 17: 185–9. PMID 9326939.