Application | Comment | Organism |
---|---|---|
medicine | the enzyme may serve as a target for manipulating NO production and treatment of NO-related diseases | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
in vitro overexpression of ASL enzyme mutant R236W in COS7 cells andprimary fibroblasts | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
R113Q | site-directed mutagenesis, the R113Q mutation abolishes the catalytic activity of the enzyme without affecting its protein stability | Homo sapiens |
R236W | site-directed mutagenesis, catalytically inactive mutant, that is structurally intact | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
cytoplasm | - |
Mus musculus | 5737 | - |
cytoplasm | - |
Homo sapiens | 5737 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
2-(Nomega-L-arginino)succinate | Mus musculus | - |
fumarate + L-arginine | - |
r | |
2-(Nomega-L-arginino)succinate | Homo sapiens | - |
fumarate + L-arginine | - |
r |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Mus musculus | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | - |
Homo sapiens | - |
liver | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
2-(Nomega-L-arginino)succinate | - |
Mus musculus | fumarate + L-arginine | - |
r | |
2-(Nomega-L-arginino)succinate | - |
Homo sapiens | fumarate + L-arginine | - |
r |
Synonyms | Comment | Organism |
---|---|---|
ASL | - |
Mus musculus |
ASL | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | argininosuccinic aciduria, ASA, MIM 207900, is the second most common human urea cycle disorder and is caused by deficiency of argininosuccinate lyase. Subjects with ASA disease cannot generate arginine from citrulline. Loss of the enzyme also leads to reduced NO synthesis due to decreased endogenous arginine synthesis as well as reduced utilization of extracellular arginine for NO production in humans | Homo sapiens |
malfunction | loss of the enzyme leads to reduced NO synthesis due to decreased endogenous arginine synthesis as well as reduced utilization of extracellular arginine for NO production in mice. Enzyme hypomorphic mice show global NO deficiency and evidence of multi-organ dysfunction, phenotypes, hypomorphic mouse model of enzyme deficiency, overview | Mus musculus |
additional information | Asl enzyme-dependent nitric oxide synthase complex | Mus musculus |
additional information | Asl enzyme-dependent nitric oxide synthase complex | Homo sapiens |
physiological function | the enzyme has a structural function in addition to its catalytic activity. The enzyme plays a more central role in cellular arginine utilization for NO synthesis beyond intracellular recycling of citrulline into arginine | Mus musculus |
physiological function | the enzyme has a structural function in addition to its catalytic activity. The enzyme plays a more central role in cellular arginine utilization for NO synthesis beyond intracellular recycling of citrulline into arginine | Homo sapiens |