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...

وصف كامل

محفوظ في:
التفاصيل البيبلوغرافية
المؤلفون الرئيسيون: Tuchman, Mendel, Caldovic, Ljubica, Daikhin, Yevgeny, Horyn, Oksana, Nissim, Ilana, Nissim, Itzhak, Korson, Mark, Burton, Barbara, Yudkoff, Marc
التنسيق: Artigo
اللغة:Inglês
منشور في: 2008
الموضوعات:
Article
الوصول للمادة أونلاين: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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