Activating Compound | Comment | Organism | Structure |
---|---|---|---|
2-hydroxyphytanoyl-CoA | - |
Homo sapiens |
Application | Comment | Organism |
---|---|---|
medicine | treatment of Refsum disease | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
expressed in Escherichia coli | Homo sapiens |
Crystallization (Comment) | Organism |
---|---|
hanging drop vapour diffusion method in 21% polyethylene glycol 3350, 0.3 M triammonium citrate, pH 7.1, at 18°C | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
E197Q | disruption of the 2-oxoglutarate binding pocket | Homo sapiens |
F275S | places a polar side chain in a hydrophobic pocket and possibly interferes with the overall structure or impair protein folding | Homo sapiens |
G204S | disruption of the 2-oxoglutarate binding pocket, uncouples 2-oxoglutarate and phytanoyl-CoA oxidation | Homo sapiens |
H259A | mutation removes two of the hydrogen bonds and probably destabilizes the beta-turn, which in turn destabilizes the core double stranded beta-helix | Homo sapiens |
I199F | disruption of the 2-oxoglutarate binding pocket | Homo sapiens |
N269H | disruption of the 2-oxoglutarate binding pocket, uncouples 2-oxoglutarate and phytanoyl-CoA oxidation | Homo sapiens |
N83Y | disruption of protein-protein interactions proposed to involve PAHX, such as that with sterol carrier protein-2, proposed to be responsible for solubilization and presentation of phytanoyl-CoA to PAHX | Homo sapiens |
R245Q | disruption of protein-protein interactions proposed to involve PAHX, such as that with sterol carrier protein-2, proposed to be responsible for solubilization and presentation of phytanoyl-CoA to PAHX | Homo sapiens |
R275Q | very low catalytic activity | Homo sapiens |
R275W | very low catalytic activity | Homo sapiens |
W193R | disruption of the 2-oxoglutarate binding pocket | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
peroxisome | - |
Homo sapiens | 5777 | - |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Fe2+ | - |
Homo sapiens |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
35440 | - |
calculated from amino acid sequence without N-terminal methionine | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | O14832 | - |
- |
Purification (Comment) | Organism |
---|---|
carboxymethyl-Sepharose cation exchange chromatography and Superdex S75 gel filtration | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
isovaleryl-CoA + 2-oxoglutarate + O2 | - |
Homo sapiens | ? | - |
? | |
phytanoyl-CoA + 2-oxoglutarate + O2 | - |
Homo sapiens | 2-hydroxyphytanoyl-CoA + succinate + CO2 | - |
ir |
Synonyms | Comment | Organism |
---|---|---|
PAHX | - |
Homo sapiens |
phytanoyl-CoA 2-hydroxylase | - |
Homo sapiens |