An enzyme of the urea cycle that catalyzes the formation of argininosuccinic acid from citrulline and aspartic acid in the presence of ATP. Absence or deficiency of this enzyme causes the metabolic disease CITRULLINEMIA in humans. EC 6.3.4.5.
Synonyms: Synthetase, Argininosuccinate, L-Citrulline:L-aspartate ligase (AMP-forming), Argininosuccinate Synthetase, Synthase, Argininosuccinate, Argininosuccinate Synthase, EC 6.3.4.5
Instance information
comment
/defic: consider also CITRULLINEMIA ,98; see ARGININOSUCCINATE SYNTHETASE 1991-97; see LIGASES 1975-90 ,98(75)
identifier
D001124