Cloned (Comment) | Organism |
---|---|
expression of His-tagged GBBH in Escherichia coli strain WK6 | Homo sapiens |
Crystallization (Comment) | Organism |
---|---|
purified recombinant His-tagged GBBH, and SeMet-labeled variant, by sitting drop vapor diffusion technique, mixing 0.001 ml of 7 mg/ml protein in 20 mM Tris-HCl, pH 8.0, with 0.001 ml of reservoir solution containing 1.2 M ammonium sulfate and 100 mM sodium acetate, pH 4.5. In co-crystallization trials with ligands, each ligand is added to the reservoir solution to a final concentration of 5 mM, X-ray diffraction structure determination and analysis at 2.0 A and 3.5 A resolution, respectively | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
3-(2,2,2-trimethylhydrazinium propionate dihydrate) | i.e. mildronate, a synthetic inhibitor of GBBH, is a non-hydroxylatable analog of gamma-butyrobetaine | Homo sapiens | |
4-Trimethylammoniobutanoate | substrate inhibition at concentrations above 0.2 mM | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.0275 | - |
4-Trimethylammoniobutanoate | pH 7.0, 37°C, recombinant enzyme | Homo sapiens |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Fe2+ | Fe(II)-binding residues form a conserved His-X-Asp-Xn-His triad | Homo sapiens | |
Zn2+ | quantitative determination of Zn2+ by atomic absorption spectrometry, zinc-binding site structure in the N-terminal domain of GBBH, overview | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-trimethylammoniobutanoate + 2-oxoglutarate + O2 | Homo sapiens | - |
3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2 | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | O75936 | - |
- |
Purification (Comment) | Organism |
---|---|
recombinant His-tagged GBBH from Escherichia coli strain WK6 by nickel affinity chromatography | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-trimethylammoniobutanoate + 2-oxoglutarate + O2 | - |
Homo sapiens | 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2 | - |
? | |
4-trimethylammoniobutanoate + 2-oxoglutarate + O2 | catalytic domain and active site structure, overview | Homo sapiens | 3-hydroxy-4-trimethylammoniobutanoate + succinate + CO2 | - |
? |
Subunits | Comment | Organism |
---|---|---|
dimer | the GBBH monomer consists of a catalytic double-stranded beta-helix domain, and a smaller N-terminal domain, the N-terminal domain of GBBH has a similar fold to the DUF971 superfamily. GBBH is dimeric in its biological state. the dimerization interface of GBBH is very different from that of related enzymes, three-dimensional structure, overview | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
gamma-butyrobetaine hydroxylase | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
7 | - |
assay at | Homo sapiens |
IC50 Value | IC50 Value Maximum | Comment | Organism | Inhibitor | Structure |
---|---|---|---|---|---|
0.062 | - |
pH 7.0, 37°C, recombinant enzyme | Homo sapiens | 3-(2,2,2-trimethylhydrazinium propionate dihydrate) |
General Information | Comment | Organism |
---|---|---|
evolution | the GBBH residues involved in zinc binding are evolutionary conserved in all sequenced eukaryotes more complex than Caenorhabditis elegans | Homo sapiens |
physiological function | GBBH is a 2-ketoglutarate-dependent dioxygenase that catalyzes the biosynthesis of L-carnitine by hydroxylation of gamma-butyrobetaine. L-Carnitine is required for the transport of long-chain fatty acids into mitochondria for generating metabolic energy | Homo sapiens |