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New Insights in Nutritional Management and Amino Acid Supplementation in Urea Cycle Disorders
Sodium phenylbutyrate is used in the pharmacological treatment of urea cycle disorders to create alternative pathways for nitrogen excretion. The primary metabolite, phenylacetate, conjugates glutamine in the liver and kidney to form phenylacetylglutamine that is readily excreted in the urine. Patie...
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| Publicat a: | Mol Genet Metab |
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| Autor principal: | |
| Format: | Artigo |
| Idioma: | Inglês |
| Publicat: |
2010
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| Matèries: | |
| Accés en línia: | https://ncbi.nlm.nih.gov/pmc/articles/PMC4831209/ https://ncbi.nlm.nih.gov/pubmed/20299258 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2010.02.019 |
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