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Literature summary extracted from

  • Searls, T.; Butler, D.; Chien, W.; Mukherji, M.; Lloyd, M.D.; Schofield, C.J.
    Studies on the specificity of unprocessed and mature forms of phytanoyl-CoA 2-hydroxylase and mutation of the iron binding ligands (2005), J. Lipid Res., 46, 1660-1667.
    View publication on PubMed

Activating Compound

EC Number Activating Compound Comment Organism Structure
1.14.11.18 ATP at 0.1 mM Fe2+ 4 mM ATP inhibits enzyme activity. At increased Fe2+ concentrations ATP stimulates with a maximum at 64 mM ATP for 1.0 mM Fe2+ Homo sapiens
1.14.11.18 GTP at 0.1 mM Fe2+ 4 mM GTP inhibits enzyme activity. At increased Fe2+ concentrations ATP stimulates with a maximum at 64 mM GTP for 1.0 mM Fe2+ Homo sapiens
1.14.11.18 imidazole at 0.1 mM Fe2+ 4 mM ATP inhibits enzyme activity. At increased Fe2+ concentrations ATP stimulates with a maximum at 64 mM ATP for 1.0 mM Fe2+ Homo sapiens
1.14.11.18 imidazole stimulates at 1.0 mM Fe2+ Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
1.14.11.18 D177A no activity with phytanoyl-CoA Homo sapiens
1.14.11.18 H175A no activity with phytanoyl-CoA Homo sapiens
1.14.11.18 H213A insoluble mutant enzyme Homo sapiens
1.14.11.18 H264A activity with phytanoyl-CoA is 7.5 of the wild-type activity Homo sapiens
1.14.11.18 Q176A activity with phytanoyl-CoA is 16.3% of the wild-type activity Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
1.14.11.18 ATP at 0.1 mM Fe2+ 4 mM ATP inhibits enzyme activity. At increased Fe2+ concentrations ATP stimulates with a maximum at 64 mM ATP for 1.0 mM Fe2+ Homo sapiens
1.14.11.18 GTP at 0.1 mM Fe2+ 4 mM ATP inhibits enzyme activity. At increased Fe2+ concentrations ATP stimulates with a maximum at 64 mM ATP for 1.0 mM Fe2+ Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
1.14.11.18 peroxisome
-
 Homo sapiens 5777
-

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
1.14.11.18 Iron Fe2+ required. Maximal activity is 0.05-0.1 mM Fe2+ in absence of ATP Homo sapiens

Organism

EC Number Organism UniProt Comment Textmining
1.14.11.18 Homo sapiens O14832
-
-

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
1.14.11.18 proteolytic modification unprocessed proenzyme contains a N-terminal peroxisomal targeting sequence that is cleaved to give mature PAHX. Both forms are able to hydroxylate a range of CoA derivatives, but under the same assay conditions, the N-terminal Hexa-His-tagged unprocessed form is less active than the nontagged mature form Homo sapiens

Purification (Commentary)

EC Number Purification (Comment) Organism
1.14.11.18
-
 Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
1.14.11.18 PAHX
-
 Homo sapiens