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    Chapter

    N-Acetylglutamate Synthetase (NAGS) Deficiency : Diagnosis, Clinical Observations and Treatment

    NAGS plays an important role in the short term regulation of urea synthesis. This enzyme has extensively been studied by Shigesada and Tatibana in rodents 1 ,2,3. We have purified human NAGS in order to find out ...

    C. Bachmann, J. P. Colombo, K. Jaggi in Urea Cycle Diseases (1982)

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    Chapter

    Orotic Acid in Urine and Hyperammonemia

    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 mitocho...

    C. Bachmann, J. P. Colombo in Urea Cycle Diseases (1982)