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D001124Level 6

Argininosuccinate Synthase

**Definition:** 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.

**Tree numbers:** - D08.811.464.259.300

**Synonyms:** - Argininosuccinate Synthetase

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