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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Detalhes bibliográficos
Main Authors: Brunetti-Pierri, Nicola, Lanpher, Brendan, Erez, Ayelet, Ananieva, Elitsa A., Islam, Mohammad, Marini, Juan C., Sun, Qin, Yu, Chunli, Hegde, Madhuri, Li, Jun, Wynn, R. Max, Chuang, David T., Hutson, Susan, Lee, Brendan
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2011
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Articles
Acesso em linha: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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