Guanidines pp 149-158 | Cite as

Arginine Metabolism in Citrullinemic Patients

  • Takeyori Saheki
  • Mariko Sase
  • Kyoko Nakano
  • Yukio Yagi


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.


Liver Kidney Arginine Metabolism Enzyme Abnormality Urea Cycle Disorder Argininosuccinate Synthetase 
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Copyright information

© Springer Science+Business Media New York 1985

Authors and Affiliations

  • Takeyori Saheki
    • 1
  • Mariko Sase
    • 1
  • Kyoko Nakano
    • 1
  • Yukio Yagi
    • 1
  1. 1.Department of Biochemistry, School of MedicineKagoshima UniversityKagoshimaJapan

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