Orotic Acid in Urine and Hyperammonemia

  • C. Bachmann
  • J. P. Colombo
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 153)


Orotoc acid (OA) an intermediary metabolite of the pyrimidine synthesis pathway is formed from carbamylphosphate (CP) and aspartate (Fig. 1). In the liver these precursors are synthetised mainly in the mitochondrium1,2,3 . Natale and Tremblay4 showed that CP formed by carbamylphosphate synthetase (CPS I) in the mitochondria crosses the mitochondrial membrane into the cytosol where the condensation with aspartate occurs. In addition especially in extrahepatic, fetal or regenerating tissues a cytosolic glutamine dependant carbamylphosphate synthetase (CPS II) exists5,6. Tremblay et al. showed that the synthesis of CP in the mitochondria accounts for a major part of the CP utilized for pyrimidine synthesis by rat liver slices.


OROTIC Acid Organic Aciduria Uric Acid Excretion Urea Cycle Disorder Propionic Acidemia 
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Copyright information

© Springer Science+Business Media New York 1982

Authors and Affiliations

  • C. Bachmann
    • 1
  • J. P. Colombo
    • 1
  1. 1.Dept. Clinical Chemistry InselspitalUniversity of BerneSwitzerland

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