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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Main Authors: Tuchman, Mendel, Caldovic, Ljubica, Daikhin, Yevgeny, Horyn, Oksana, Nissim, Ilana, Nissim, Itzhak, Korson, Mark, Burton, Barbara, Yudkoff, Marc
Format: Artigo
Jezik:Inglês
Izdano: 2008
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Article
Online dostop: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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