Citrullinemia

In their exceptionally interesting article, "Citrullinemia with defective urea production,"1 the authors discuss the metabolism of arginine in their citrullinemic patient: When given for a sustained period, arginine increased the production of urea stoichiometrically but equi-molar amounts...

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Veröffentlicht in:Pediatrics (Evanston) 1968-03, Vol.41 (3), p.690-690
Hauptverfasser: Morrow, G, Barness, L A
Format: Artikel
Sprache:eng
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Zusammenfassung:In their exceptionally interesting article, "Citrullinemia with defective urea production,"1 the authors discuss the metabolism of arginine in their citrullinemic patient: When given for a sustained period, arginine increased the production of urea stoichiometrically but equi-molar amounts of ornithine, citrulline, argininosuccinic acid, and arginine were not recovered from the urine. . . . Nitrogen balances in addition to protein and arginine loading studies suggest alternate pathways for the degradation of arginine. According to our investigations2, considerable activity of ornithine-ketoacid aminotransferase (EC 2.6.1.13) can be demonstrated in human liver tissue.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.41.3.690