argininosuccinate synthase
Oxford Dictionary of Biochemistry:
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argininosuccinate synthase
EC 6.3.4.5; systematic name: L-citrulline:L-aspartate ligase (AMP-forming); other names: argininosuccinate synthetase; citrulline-aspartate ligase. An enzyme of the ornithine-urea cycle that catalyses a reaction between ATP, L-citrulline, and L-aspartate to form L-argininosuccinate with release of AMP and pyrophosphate. The human enzyme is a cytosolic homotetramer expressed mainly in hepatocytes. More than 20 mutations in a locus at 9q34 result in enzyme deficiency with consequent hypercitrullinemia and hyperammonemia.
| argininosuccinate, argininosuccinase, arginino | |
| arginyl, argon detector, arm |
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Oxford Dictionary of Biochemistry
Oxford University Press. Oxford Dictionary of Biochemistry and Molecular Biology © 1997, 2000, 2006 All rights reserved.
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