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]