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Proteins/UNIPROT:P04424
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. 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..
Function from UniProtKB (P04424), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 1 · substrate of
1 compound
1 compound.
0 reported routes1 assembled route
1 row
224 statements
224 statements · 8 reference databases · Subject: ASL · Sources: Bgee, gene ontology, Hetionet, Human Protein Atlas, Human-GEM, Reactome, Rhea, WikiPathways
2 reactions
L-arginine+fumaric acid2-(N(omega)-L-arginino)succinate
Catalysed by ASL
Reaction pageReversibleBalancedEC 4.3.2.1cytosol
Human-GEM MAR03813CC BY 4.0Reference data
2-(N(omega)-L-arginino)succinatefumaric acid+L-arginine
Catalysed by ASL
Reaction pageReversibleBalanced
Rhea RHEA:24020CC BY 4.0Reference data
2 names
Resolves to: ASL