Cloned (Comment) | Organism |
---|---|
expression of N-terminally His-tagged wild-type and mutant enzymes in Escherichia coli strain Rosetta 2(DE3)pLysS | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
A219V | site-directed mutagenesis, inactive mutant | Homo sapiens |
R303C | naturally occuring mutation, causing the mild form, Type II, of enzyme deficiency, the mutant shows reduced activity compared to the wild-type enzyme, mutation R303C increases the KM value | Homo sapiens |
R426H | naturally occuring mutation, causing the severe form, Type I, of enzyme deficiency, the mutant shows reduced activity compared to the wild-type enzyme, mutation R426H reduces the KM value | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
additional information | - |
additional information | non-linear dependence of the activities on the substrate ratios due to competitive binding. The enzyme does not follow simple Michaelis-Menten kinetics, kinetic analysis of wild-type and mutant enzymes, overview. Wild-type enzyme and mutant R426H appear to have equivalent cooperative binding on both substrates, while mutant R303C demonstrates no cooperativity | Homo sapiens | |
0.0007 | - |
phosphoribosylsuccinyl-aminoimidazole carboxamide | recombinant His-tagged mutant R426H, pH 7.4, 25°C | Homo sapiens | |
0.0014 | - |
succinyladenosine monophosphate | recombinant His-tagged mutant R426H, pH 7.4, 25°C | Homo sapiens | |
0.00174 | - |
phosphoribosylsuccinyl-aminoimidazole carboxamide | recombinant His-tagged wild-type enzyme, pH 7.4, 25°C | Homo sapiens | |
0.0021 | - |
succinyladenosine monophosphate | recombinant His-tagged wild-type enzyme, pH 7.4, 25°C | Homo sapiens | |
0.0027 | - |
succinyladenosine monophosphate | recombinant His-tagged mutant R303C, pH 7.4, 25°C | Homo sapiens | |
0.009 | - |
phosphoribosylsuccinyl-aminoimidazole carboxamide | recombinant His-tagged mutant R303C, pH 7.4, 25°C | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
phosphoribosylsuccinyl-aminoimidazole carboxamide | Homo sapiens | - |
phosphoribosylaminoimidazole carboxamide + fumarate | - |
? | |
succinyladenosine monophosphate | Homo sapiens | - |
AMP + fumarate | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Purification (Comment) | Organism |
---|---|
recombinant His-tagged wild-type and mutant enzymes from Escherichia coli strain Rosetta 2(DE3)pLysS by nickel affinity chromatography | Homo sapiens |
Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|
6.4 | - |
purified recombinant His-tagged mutant R426H enzyme, substrate phosphoribosylsuccinyl-aminoimidazole carboxamide, pH 7.4, 25°C | Homo sapiens |
6.7 | - |
purified recombinant His-tagged mutant R426H enzyme, substrate succinyladenosine monophosphate, pH 7.4, 25°C | Homo sapiens |
13.9 | - |
purified recombinant His-tagged wild-type enzyme, substrate succinyladenosine monophosphate, pH 7.4, 25°C | Homo sapiens |
23.3 | - |
purified recombinant His-tagged wild-type enzyme, substrate phosphoribosylsuccinyl-aminoimidazole carboxamide, pH 7.4, 25°C | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
phosphoribosylsuccinyl-aminoimidazole carboxamide | - |
Homo sapiens | phosphoribosylaminoimidazole carboxamide + fumarate | - |
? | |
succinyladenosine monophosphate | - |
Homo sapiens | AMP + fumarate | - |
? |
Subunits | Comment | Organism |
---|---|---|
homotetramer | - |
Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
ADSL | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
25 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
7.4 | - |
assay at | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | missense mutations in the protein lead to enzyme deficiency, an inborn error of purine metabolism characterized by neurological and physiological symptoms. Enzyme deficiency is biochemically diagnosed by elevated levels of succinylaminoimidazolecarboxamide riboside and succinyladenosine, the dephosphorylated derivatives of the substrates. Succinyladenosine/succinylaminoimidazolecarboxamide riboside ratios are associated with three phenotypic groups | Homo sapiens |
metabolism | adenylosuccinate lyase is involved in the de novo purine biosynthesis pathway and purine nucleotide cycle | Homo sapiens |
kcat/KM Value [1/mMs-1] | kcat/KM Value Maximum [1/mMs-1] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
915 | - |
succinyladenosine monophosphate | recombinant His-tagged mutant R303C, pH 7.4, 25°C | Homo sapiens | |
3640 | - |
phosphoribosylsuccinyl-aminoimidazole carboxamide | recombinant His-tagged mutant R303C, pH 7.4, 25°C | Homo sapiens | |
17800 | - |
succinyladenosine monophosphate | recombinant His-tagged mutant R426H, pH 7.4, 25°C | Homo sapiens | |
25200 | - |
succinyladenosine monophosphate | recombinant His-tagged wild-type enzyme, pH 7.4, 25°C | Homo sapiens | |
37500 | - |
phosphoribosylsuccinyl-aminoimidazole carboxamide | recombinant His-tagged mutant R426H, pH 7.4, 25°C | Homo sapiens | |
50900 | - |
phosphoribosylsuccinyl-aminoimidazole carboxamide | recombinant His-tagged wild-type enzyme, pH 7.4, 25°C | Homo sapiens |