Application | Comment | Organism |
---|---|---|
medicine | high doses of pyridoxine could be an effective therapy for cystathioninuric patients harboring the T67I mutation and be somewhat less effective in ameliorating the symptoms associated with the Q240E mutation | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
expressed in Escherichia coli | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
Q240E | missense mutation. Exhibits a 70fold decrease in Vmax compared to that of wild-type CGL. The KMs for L-cystathionine are comparable to that of wild type CGL. The pyridoxal 5'-phosphate content of the Q240E mutant is about 80fold lower than that of wild-type enzyme | Homo sapiens |
T67I | missense mutation. Exhibits a 3.5fold decrease in Vmax compared to that of wild-type CGL. The KMs for L-cystathionine are comparable to that of wild type CGL. The pyridoxal 5'-phosphate content of the T67I mutant is about 4fold lower than that of wild-type enzyme | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.4 | - |
L-cystathionine | for variant S403 | Homo sapiens | |
0.6 | - |
L-cystathionine | for mutant T67I | Homo sapiens | |
0.6 | - |
L-cystathionine | for variant I403 | Homo sapiens | |
0.72 | - |
L-cystathionine | for mutant Q240E | Homo sapiens | |
3.4 | - |
L-cysteine | for variant I403 | Homo sapiens | |
3.5 | - |
L-cysteine | for variant S403 | Homo sapiens | |
4.1 | - |
L-cysteine | for mutant T67I | Homo sapiens | |
5.4 | - |
L-homocysteine | for variant S403 | Homo sapiens | |
7 | - |
L-homocysteine | for variant I403 | Homo sapiens | |
8 | - |
L-homocysteine | for mutant T67I | Homo sapiens |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
156000 | - |
T67I CGL mutant | Homo sapiens |
156000 | - |
wild-type, estimated for CGL from its retention time of 42.8 min | Homo sapiens |
170000 | - |
Q240E CGL mutant | Homo sapiens |
178000 | - |
wild-type, molecular masses of both the T67I CGL (156 kDa) and the Q240E mutant (170 kDa) are comparable to that of wild-type CGL (167 kDa), indicating that both mutants exist as tetrameric proteins | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | P32929 | - |
- |
Purification (Comment) | Organism |
---|---|
combination of Ni-NTA chromatography, anion exchange chromatography, and gel filtration steps | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
L-cystathionine + H2O | - |
Homo sapiens | L-cysteine + 2-oxobutanoate + NH3 | - |
? | |
L-cysteine + H2O | - |
Homo sapiens | sulfide + NH3 + pyruvate | - |
? | |
L-homocysteine | - |
Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
homotetramer | In the three-dimensional structure of human CGL, two monomers contribute residues to each of the two active sites in the dimer, and two dimers come together to form a tetramer | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
cgl | CGL variant S403 and I403, polymorphic variants at amino acid residues 403 | Homo sapiens |
human cystathionine-gamma-lyase | pyridoxal-5'-phosphate (PLP)-dependent enzyme | Homo sapiens |
Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.02 | - |
L-cystathionine | for mutante Q240E | Homo sapiens | |
0.3 | - |
L-cysteine | for mutante T67I | Homo sapiens | |
0.45 | - |
L-cystathionine | for mutante T67I | Homo sapiens | |
0.67 | - |
L-cysteine | for variante I403 | Homo sapiens | |
0.67 | - |
L-cysteine | for variante S403 | Homo sapiens | |
0.82 | - |
L-homocysteine | for mutante T67I | Homo sapiens | |
1.7 | - |
L-cystathionine | for variante S403 | Homo sapiens | |
1.9 | - |
L-cystathionine | for variante I403 | Homo sapiens | |
3.5 | - |
L-homocysteine | for variante S403 | Homo sapiens | |
3.7 | - |
L-homocysteine | for variante I403 | Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
pyridoxal 5'-phosphate | - |
Homo sapiens |