Metabolism of Compounds Labeled with 15N by an Infant with Congenital Hyperammonemia

A patient with argininosuccinc aciduria was charged with 50 grams of protein, which was followed by considerable hyperammonemia. There was no response in further urea formation; but there was a considerable production of orotic acid, a precursor of pyrimidines. This makes orotic acid to an important diagnostic tool for the diagnosis of impaired urea formation. The patient's plasma amino acid pattern led to the suggestion that orotic acid synthesis is initiated by increased de novo formation of carbamyl phosphate in the cytosol and not by deviation of already existing intramitochondrial carbamyl phosphate.

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Influence of testosterone on urinary 15N-balance in normal subjects and patients with testicular feminization

Zachmann

Zagalak

Völlmin

et al. 1977

Clinica Chimica Acta

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Metabolism of Compounds Labeled with 15N by an Infant with Congenital Hyperammonemia

Cited by 9 publications

References 14 publications

A selected bibliography of biomedical and environmental applications of stable isotopes. III—15N 1971-1976

A selected bibliography of biomedical and environmental applications of stable isotopes. III—15N 1971-1976

Protein load in argininosuccinic aciduria: thoughts on its biochemical implications

Influence of testosterone on urinary 15N-balance in normal subjects and patients with testicular feminization

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