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N-carbamylglutamate Markedly Enhances Ureagenesis in N-acetylglutamate Deficiency and Propionic Acidemia as Measured by Isotopic Incorporation and Blood Biomarkers

N-acetylglutamate (NAG) is an endogenous essential cofactor for conversion of ammonia to urea in the liver. Deficiency of NAG causes hyperammonemia and occurs because of inherited deficiency of its producing enzyme, NAG synthase (NAGS), or interference with its function by short fatty acid derivativ...

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מידע ביבליוגרפי
Main Authors: Tuchman, Mendel, Caldovic, Ljubica, Daikhin, Yevgeny, Horyn, Oksana, Nissim, Ilana, Nissim, Itzhak, Korson, Mark, Burton, Barbara, Yudkoff, Marc
פורמט: Artigo
שפה:Inglês
יצא לאור: 2008
נושאים:
גישה מקוונת:https://ncbi.nlm.nih.gov/pmc/articles/PMC2640836/
https://ncbi.nlm.nih.gov/pubmed/18414145
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1203/PDR.0b013e318179454b
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