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Details on Person UniProt:P04424-2 ASL
| Class:Id | ReferenceIsoform:8968679 |
|---|---|
| _chainChangeLog | initiator methionine:1 added on Fri February 17 2017;chain:2-464 added on Fri February 17 2017;initiator methionine:1 for 8968679 removed on Fri Nov 03 2023;initiator methionine: for 8968679 added on Fri Nov 03 2023;initiator methionine: for 8968679 removed on Fri Aug 15 2025;initiator methionine:1 for 8968679 added on Fri Aug 15 2025 |
| _displayName | UniProt:P04424-2 ASL |
| _timestamp | 2025-08-15 20:58:08 |
| chain | initiator methionine:1 chain:2-464 |
| checksum | F751625C1A581883 |
| comment | FUNCTION Catalyzes the reversible cleavage of L-argininosuccinate to fumarate and L-arginine, an intermediate step reaction in the urea cycle mostly providing for hepatic nitrogen detoxification into excretable urea as well as de novo L-arginine synthesis in nonhepatic tissues (PubMed:11747432, PubMed:11747433, PubMed:22081021, PubMed:2263616, PubMed:9045711). Essential regulator of intracellular and extracellular L-arginine pools. As part of citrulline-nitric oxide cycle, forms tissue-specific multiprotein complexes with argininosuccinate synthase ASS1, transport protein SLC7A1 and nitric oxide synthase NOS1, NOS2 or NOS3, allowing for cell-autonomous L-arginine synthesis while channeling extracellular L-arginine to nitric oxide synthesis pathway (PubMed:22081021).CATALYTIC ACTIVITY 2-(N(omega)-L-arginino)succinate = fumarate + L-arginineACTIVITY REGULATION Enzyme activity is regulated by acetylation.BIOPHYSICOCHEMICAL PROPERTIES Amino-acid biosynthesis; L-arginine biosynthesis; L-arginine from L-ornithine and carbamoyl phosphate: step 3/3.PATHWAY Nitrogen metabolism; urea cycle; L-arginine and fumarate from (N(omega)-L-arginino)succinate: step 1/1.SUBUNIT Homotetramer (PubMed:11747433). Forms tissue-specific complexes with ASS1, SLC7A1, HSP90AA1 and nitric oxide synthase NOS1, NOS2 or NOS3; the complex maintenance is independent of ASL catalytic function (By similarity).INTERACTION Acetylation modifies enzyme activity in response to alterations of extracellular nutrient availability. Acetylation increased with trichostin A (TSA) or with nicotinamide (NAM). Glucose increases acetylation by about a factor of 3 with decreasing enzyme activity. Acetylation on Lys-288 is decreased on the addition of extra amino acids resulting in activation of enzyme activity.DISEASE The disease is caused by variants affecting the gene represented in this entry. The phenotype heterogeneity among patients is associated with interallelic complementation resulting in either complete loss of activity or partial regeneration of functional active sites in the heterotetrameric mutant protein.SIMILARITY Belongs to the lyase 1 family. Argininosuccinate lyase subfamily. |
| created | [InstanceEdit:8964659] Weiser, JD |
| description | recommendedName: fullName evidence="15"Argininosuccinate lyase shortName: ASAL ecNumber evidence="3 4 14"4.3.2.1 alternativeName: Arginosuccinase |
| geneName | ASL |
| identifier | P04424 |
| isoformParent | |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation Alternative splicing Amino-acid biosynthesis Arginine biosynthesis Disease variant Lyase Proteomics identification Reference proteome Urea cycle |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9963647] Weiser, Joel, 2025-08-15 |
| name | ASL |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:9002806] ENSEMBL:ENSG00000126522 ASL [Homo sapiens] |
| secondaryIdentifier | ARLY_HUMAN E7EMI0 E9PE48 Q6LDS5 Q96HS2 |
| sequenceLength | 464 |
| species | [Species:48887] Homo sapiens |
| variantIdentifier | P04424-2 |
| [Change default viewing format] | |
No pathways have been reviewed or authored by UniProt:P04424-2 ASL (8968679)
