EC Number | Activating Compound | Comment | Organism | Structure |
---|---|---|---|---|
1.14.11.18 | albumin | 0.05 mM 3-methylhexadecanoyl-CoA, no hydroxylation in absence of albumin. Hydroxylation of 3-methylnonanoyl-CoA or 3-methyldodecanoyl-CoA is much less dependent on the presence of albumin | Homo sapiens |
EC Number | KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|---|
1.14.11.18 | 0.0408 | - |
3-methylhexadecanoyl-CoA | - |
Homo sapiens |
EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
1.14.11.18 | additional information | Homo sapiens | Resum disease might be characterized by an accumulation of not only phytanic acid but also ther 3-alkyl-branched fatty acids | ? | - |
? |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
1.14.11.18 | Homo sapiens | - |
- |
- |
EC Number | Purification (Comment) | Organism |
---|---|---|
1.14.11.18 | - |
Homo sapiens |
EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
1.14.11.18 | 3,7-dimethyloctan-6-enoyl-CoA + 2-oxoglutarate + O2 | 75.2 of the activity with 3-methylhexadecanoyl-CoA | Homo sapiens | 3,7-dimethyl-2-hydroxyocta-7-enoyl-CoA + succinate + CO2 | - |
? | |
1.14.11.18 | 3-ethylnonanoyl-CoA + 2-oxoglutarate + O2 | 64.7% of the activity with 3-methylhexadecanoyl-CoA | Homo sapiens | 3-ethoxy-2-hydroxyhexadecanoyl-CoA + succinate + CO2 | - |
? | |
1.14.11.18 | 3-methyl-5-phenyl-pentanoyl-CoA + 2-oxoglutarate + O2 | 53% of the activity with 3-methylhexadecanoyl-CoA | Homo sapiens | 2-hydroxy-3-methyl-5-phenylpentanoyl-CoA + succinate + CO2 | - |
? | |
1.14.11.18 | 3-methyldodecanoyl-CoA + 2-oxoglutarate + O2 | 110% of the activity with 3-methylhexadecanoyl-CoA | Homo sapiens | 2-hydroxy-3-methyldodecanoyl-CoA + succinate + CO2 | - |
? | |
1.14.11.18 | 3-methylheptanoyl-CoA + 2-oxoglutarate + O2 | 16.2% of the activity with 3-methylhexadecanoyl-CoA | Homo sapiens | 2-hydroxy-3-methylheptanoyl-CoA + succinate + CO2 | - |
? | |
1.14.11.18 | 3-methylhexadecanoyl-CoA + 2-oxoglutarate + O2 | - |
Homo sapiens | 2-hydroxy-3-methylhexadecanoyl-CoA + succinate + CO2 | - |
? | |
1.14.11.18 | 3-methylnonanoyl-CoA + 2-oxoglutarate + O2 | 111% of the activity with 3-methylhexadecanoyl-CoA | Homo sapiens | 2-hydroxy-3-methylnonanoyl-CoA + succinate + CO2 | - |
? | |
1.14.11.18 | 3-methylundecanoyl-CoA + 2-oxoglutarate + O2 | 110% of the activity with 3-methylhexadecanoyl-CoA | Homo sapiens | 2-hydroxy-3-methylundecanoyl-CoA + succinate + CO2 | - |
? | |
1.14.11.18 | additional information | Resum disease might be characterized by an accumulation of not only phytanic acid but also ther 3-alkyl-branched fatty acids | Homo sapiens | ? | - |
? | |
1.14.11.18 | additional information | no activity with isovaleryl-CoA, 4-phenyl-3-methylbutanoyl-CoA, 3-methylpentanoyl-CoA, hexadecanoyl-CoA, octanoyl-CoA, prostanoyl-CoA or 4-methylnonaoyl-CoA. Chain length of at least seven carbon atoms is a prerequisite for PAHX-dependent hydroxylation. The optimal chain length appears to be 9-12 carbon atoms. The branch at position 3 is a prerequisite for the activity of PAHX | Homo sapiens | ? | - |
? | |
1.14.11.18 | phytanoyl-CoA + 2-oxoglutarate + O2 | - |
Homo sapiens | 2-hydroxyphytanoyl-CoA + succinate + CO2 | - |
? |
EC Number | Synonyms | Comment | Organism |
---|---|---|---|
1.14.11.18 | PAHX | - |
Homo sapiens |