argininosuccinate synthase

argininosuccinate synthase
ar·gi·ni·no·suc·cin·ate syn·thase (ahr″jĭ-ne″no-sukґsĭ-nāt sinґthās) [EC 6.3.4.5] an enzyme of the ligase class that catalyzes the condensation of citrulline and aspartate to form argininosuccinate. The reaction is a part of the urea cycle in the liver (see illustration at urea cycle, under cycle). Deficiency of enzyme activity causes citrullinemia. Written also argininosuccinate synthetase.

Medical dictionary. 2011.

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  • citrullinemia — Urea cycle disorder in which citrulline concentrations in the blood, urine, and cerebrospinal fluid are elevated, because of deficiency of arginosuccinate synthetase (ASS); manifested clinically by lethargy …   Medical dictionary

  • urea cycle — a series of metabolic reactions, occurring in the liver, by which ammonia is converted to urea using cyclically regenerated ornithine as a carrier. See illustration. Urea cycle. Diagrammatic representation of reactions by which excess nitrogen in …   Medical dictionary

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  • Urea cycle — The urea cycle (also known as the ornithine cycle) is a cycle of biochemical reactions occurring in many animals that produces urea ((NH2)2CO) from ammonia (NH3). This cycle was the first metabolic cycle discovered (Hans Krebs and Kurt Henseleit …   Wikipedia

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