Cloned (Comment) | Organism |
---|---|
catalytic subunit DNA sequence determination and analysis, expression as His-tagged wild-type enzyme and mutant R127C in Rosetta cells, functional expression of wild-type and mutant enzyme in enzyme-deficient cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
R127C | naturally occuring mutation in patients with hemolytic anemia resulting from low erythrocyte enzyme levels, fibroblast cells from these patients bearing the mutation show 95% reduced enzyme activity and lowered Km for glutamate and aminobutyrate, as well as an increased Ki for GSH | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
GSH | feedback inhibition, mutant R127C is not sensitive | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.25 | - |
L-alpha-aminobutyrate | mutant R127C, pH 8.0, 37°C | Homo sapiens | |
0.63 | - |
L-Glu | mutant R127C, pH 8.0, 37°C | Homo sapiens | |
2.39 | - |
L-alpha-aminobutyrate | wild-type enzyme, pH 8.0, 37°C | Homo sapiens | |
7.16 | - |
L-Glu | wild-type enzyme, pH 8.0, 37°C | Homo sapiens |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Mg2+ | - |
Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + L-Glu + L-Cys | Homo sapiens | first and rate limiting step in GSH biosynthesis, rare hereditary enzyme deficiency is associated with low erythrocyte levels of the enzyme leading to hemolytic anemia | ADP + phosphate + gamma-L-Glu-L-Cys | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Purification (Comment) | Organism |
---|---|
recombinant His-tagged wild-type and mutant R127C enzyme from Rosetta cells | Homo sapiens |
Reaction | Comment | Organism | Reaction ID |
---|---|---|---|
ATP + L-glutamate + L-cysteine = ADP + phosphate + gamma-L-glutamyl-L-cysteine | substrate binding mechanism and catalytic site structure determination and analysis, Arg127 and conserved Cys249 are involved | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | dermal | Homo sapiens | - |
Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|
0.44 | - |
purified mutant R127C enzyme, substrates L-glutamate and L-alpha-aminobutyrate | Homo sapiens |
8.86 | - |
purified wild-type enzyme, substrates L-glutamate and L-alpha-aminobutyrate | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + L-Glu + L-alpha-aminobutyrate | - |
Homo sapiens | ADP + phosphate + gamma-L-Glu-L-alpha-aminobutyrate | - |
? | |
ATP + L-Glu + L-Cys | - |
Homo sapiens | ADP + phosphate + gamma-L-Glu-L-Cys | - |
? | |
ATP + L-Glu + L-Cys | first and rate limiting step in GSH biosynthesis, rare hereditary enzyme deficiency is associated with low erythrocyte levels of the enzyme leading to hemolytic anemia | Homo sapiens | ADP + phosphate + gamma-L-Glu-L-Cys | - |
? |
Subunits | Comment | Organism |
---|---|---|
More | the holoenzyme consists of a heavy catalytic and a light modifier subunit, i.e. gamma-GCSH and gamma-GCSL, protein modeling | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
gamma-GCS | - |
Homo sapiens |
gamma-Glutamylcysteine synthetase | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
8 | - |
assay at | Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
ATP | dependent on | Homo sapiens |
Ki Value [mM] | Ki Value maximum [mM] | Inhibitor | Comment | Organism | Structure |
---|---|---|---|---|---|
0.45 | - |
GSH | wild-type enzyme, pH 8.0, 37°C | Homo sapiens | |
1000 | - |
GSH | above, mutant R127C, pH 8.0, 37°C | Homo sapiens |