Fitanoil-KoA dioksigenaza
Fitanoil-KoA dioksigenaza (EC 1.14.11.18, fitanoil-KoA hidroksilaza) je enzim sa sistematskim imenom fitanoil-KoA, 2-oksoglutarat:kiseonik oksidoreduktaza (2-hidroksilacija).[1][2][3][4][5] Ovaj enzim katalizuje sledeću hemijsku reakciju:
Fitanoil-KoA dioksigenaza | |||||||||
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Identifikatori | |||||||||
EC broj | 1.14.11.18 | ||||||||
CAS broj | 185402-46-4 | ||||||||
Baze podataka | |||||||||
IntEnz | IntEnz pregled | ||||||||
BRENDA | BRENDA pristup | ||||||||
ExPASy | NiceZyme pregled | ||||||||
KEGG | KEGG pristup | ||||||||
MetaCyc | metabolički put | ||||||||
PRIAM | profil | ||||||||
Strukture PBP | RCSB PDB PDBe PDBj PDBsum | ||||||||
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- fitanoil-KoA + 2-oksoglutarat + O2 2-hidroksifitanoil-KoA + sukcinat + CO2
Ovaj enzim je deo metaboličkog puta alfa oksidacije peroksisomalne fitanske kiseline. Za rad ovog enzima su neophodni Fe2+ i askorbat.
Reference уреди
- ^ Jansen, G.A., Mihalik, S.J., Watkins, P.A., Jakobs, C., Moser, H.W. and Wanders, R.J.A. (1998). „Characterization of phytanoyl-CoA hydroxylase in human liver and activity measurements in patients with peroxisomal disorders”. Clin. Chim. Acta. 271: 203—211. PMID 9565335.
- ^ Jansen, G.A., Mihalik, S.J., Watkins, P.A., Moser, H.W., Jakobs, C., Denis, S. and Wanders, R.J.A. (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 α-oxidation in humans”. Biochem. Biophys. Res. Commun. 229: 205—210. PMID 8954107.
- ^ 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. (1997). „Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene”. Nat. Genet. 17: 190—193. PMID 9326940.
- ^ Mihalik, S.J., Rainville, A.M. and Watkins, P.A. (1995). „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. PMID 7556205.
- ^ Mihalik, S.J., Morrell, J.C., Kim, D., Sacksteder, K.A., Watkins, P.A. and Gould, S.J. (1997). „Identification of PAHX, a Refsum disease gene”. Nat. Genet. 17: 185—189. PMID 9326939.
Literatura уреди
- Nicholas C. Price; Lewis Stevens (1999). Fundamentals of Enzymology: The Cell and Molecular Biology of Catalytic Proteins (Third изд.). USA: Oxford University Press. ISBN 019850229X.
- Eric J. Toone (2006). Advances in Enzymology and Related Areas of Molecular Biology, Protein Evolution (Volume 75 изд.). Wiley-Interscience. ISBN 0471205036.
- Branden C; Tooze J. Introduction to Protein Structure. New York, NY: Garland Publishing. ISBN 0-8153-2305-0.
- Irwin H. Segel. Enzyme Kinetics: Behavior and Analysis of Rapid Equilibrium and Steady-State Enzyme Systems (Book 44 изд.). Wiley Classics Library. ISBN 0471303097.
Spoljašnje veze уреди
- Phytanoyl-CoA+dioxygenase на US National Library of Medicine Medical Subject Headings (MeSH)