Reactome: A Curated Pathway Database

ATP + aspartate + citrulline <=> argininosuccinate + AMP + pyrophosphate (R-HSA-70577)

Species Homo sapiens


Cytosolic argininosuccinate synthase catalyzes the reaction of aspartate, citrulline, and ATP to form argininosuccinate, AMP, and pyrophosphate. The function of the human enzyme in vivo is inferred from the hypercitrullinemia observed in patients with defective forms of the enzyme (e.g., Engel et al. 2009). The enzyme is active as a homotetramer (O??Brien 1980; Karlberg et al. 2008).

Locations in the PathwayBrowser
Additional Information
Compartment cytosol
Components of this entry
Input entries
Output entries
Catalyst Activity
PhysicalEntity Activity Active Units
ASS1 tetramer argininosuccinate synthase activity (0004055)
Cross References
Database Identifier
Rhea 10933, 10935
Literature References
pubMedId Title Journal Year
19006241 Mutations and polymorphisms in the human argininosuccinate synthetase (ASS1) gene Hum Mutat 2009
18323623 Structure of human argininosuccinate synthetase Acta Crystallogr D Biol Crystallogr 2008
518841 Isolation and characterization of argininosuccinate synthetase from human liver. Biochemistry 1980