Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate.

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Caldovic L, Morizono H, Daikhin Y, Nissim I, McCarter RJ, Yudkoff M, Tuchman M

Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate.

J Pediatr. 2004 Oct;145(4):552-4.

PubMed ID
15480384 [ View in PubMed
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Abstract

In a patient with N-acetylglutamate synthase (NAGS) deficiency, incorporation of an isotopic label from ammonium chloride into urea was markedly reduced before treatment with N-carbamyl-L-glutamate (NCLG) and completely normalized following treatment. Blood ammonia rose following ammonium tracer ingestion before treatment but remained low following treatment. Serum urea concentration doubled following the treatment.

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