Protein Variants | Comment | Organism |
---|---|---|
A192V | mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W | Homo sapiens |
G280R | mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W | Homo sapiens |
R272C | mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W | Homo sapiens |
R304W | mutant G280R has no ASS activity, mutants A192V, R272C, and R304W have low ASS activity and abnormal kinetics. A higher Km for citrulline than in wild-type enzyme is obtained with mutants A192V, R272C, and R304W | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
additional information | - |
additional information | - |
Homo sapiens | |
0.027 | - |
Asp | wild-type enzyme | Homo sapiens | |
0.051 | - |
ATP | wild-type enzyme | Homo sapiens | |
0.056 | - |
citrulline | wild-type enzyme | Homo sapiens | |
0.081 | - |
ATP | mutant enzyme A192V | Homo sapiens | |
0.14 | - |
ATP | mutant R272C | Homo sapiens | |
0.44 | - |
ATP | mutant R304W | Homo sapiens | |
1.5 | - |
Asp | mutant A192V | Homo sapiens | |
4.2 | - |
citrulline | mutant R272C | Homo sapiens | |
4.3 | - |
Asp | mutant R272C | Homo sapiens | |
15 | - |
citrulline | mutant A192V | Homo sapiens | |
56 | - |
citrulline | mutant R304W | Homo sapiens | |
190 | - |
citrulline | mutant R304W | Homo sapiens |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
46000 | - |
4 * 46000, SDS-PAGE | Homo sapiens |
180000 | - |
gel filtration | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + L-citrulline + L-Asp | Homo sapiens | urea-cycle enzyme | ? | - |
? | |
additional information | Homo sapiens | citrullinemia is an autosomal recessive disease caused by a deficiency of argininosuccinate synthetase | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
wild-type and mutant enzymes | - |
Homo sapiens | - |
wild-type and mutant enzyme | - |
Homo sapiens | - |
citrullinemia is an autosomal recessive disease caused by a deficiency of argininosuccinate synthetase | - |
Purification (Comment) | Organism |
---|---|
- |
Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + citrulline + aspartate | - |
Homo sapiens | AMP + diphosphate + 2-(Nomega-L-arginino)succinate | - |
? | |
ATP + L-citrulline + L-Asp | - |
Homo sapiens | AMP + diphosphate + L-argininosuccinate | - |
? | |
ATP + L-citrulline + L-Asp | urea-cycle enzyme | Homo sapiens | ? | - |
? | |
additional information | citrullinemia is an autosomal recessive disease caused by a deficiency of argininosuccinate synthetase | Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
tetramer | 4 * 46000, SDS-PAGE | Homo sapiens |