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Phenylbutyrate therapy for maple syrup urine disease
Therapy with sodium phenylacetate/benzoate or sodium phenylbutyrate in urea cycle disorder patients has been associated with a selective reduction in branched-chain amino acids (BCAA) in spite of adequate dietary protein intake. Based on this clinical observation, we investigated the potential of ph...
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| Main Authors: | , , , , , , , , , , , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado: |
Oxford University Press
2011
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| Assuntos: | |
| Acceso en liña: | https://ncbi.nlm.nih.gov/pmc/articles/PMC3024040/ https://ncbi.nlm.nih.gov/pubmed/21098507 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddq507 |
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