Arginine Metabolism in Citrullinemic Patients
Arginine is synthesized from citrulline by the catalytic actions of argininosuccinate synthetase (ASS) and argininosuccinase in the kidney1,2, and utilized as a precursor of creatine biosynthesis or supplied to various organs for protein synthesis. The liver performing ureogenesis also synthesizes arginine, which, however, is rapidly split to ornithine and urea by the potent activity of arginase.
KeywordsLiver Kidney Arginine Metabolism Enzyme Abnormality Urea Cycle Disorder Argininosuccinate Synthetase
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