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Literature summary extracted from

  • Jansen, G.A.; Ferdinandusse, S.; Hogenhout, E.M.; Verhoeven, N.M.; Jakobs, C.; Wanders, R.J.A.
    Phytanoyl-CoA hydroxylase deficiency. Enzymological and molecular basis of classical Refsum disease (1999), Adv. Exp. Med. Biol., 466, 371-376.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
1.14.11.18 in yeast expression vector Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
1.14.11.18 Homo sapiens
-
 human
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.14.11.18 liver
-
 Homo sapiens
-
1.14.11.18 skin fibroblast
-
 Homo sapiens
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
1.14.11.18 0.0000022
-
 in fibroblast homogenate Homo sapiens
1.14.11.18 0.000041
-
 in liver homogenate Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
1.14.11.18 phytanoyl-CoA + 2-oxoglutarate + O2
-
 Homo sapiens alpha-hydroxyphytanoyl-CoA + succinate + CO2
-
 ?