Fitanoil-KoA dioksigenaza

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Fitanoil-KoA dioksigenaza
Identifikatori
EC broj 1.14.11.18
CAS broj 185402-46-4
IntEnz IntEnz view
BRENDA BRENDA entry
ExPASy NiceZyme view
KEGG KEGG entry
MetaCyc metabolic pathway
PRIAM profile
PDB structures

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 + 2-oksoglutarat + O2 \rightleftharpoons 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]

  1. 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. 
  2. 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. 
  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. (1997). „Refsum disease is caused by mutations in the phytanoyl-CoA hydroxylase gene“. Nat. Genet. 17: 190-193. PMID 9326940. 
  4. 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. 
  5. 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]

Spoljašnje veze[уреди]