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Metformin inhibits Branched Chain Amino Acid (BCAA) derived ketoacidosis and promotes metabolic homeostasis in MSUD
Maple Syrup Urine Disease (MSUD) is an inherited disorder caused by the dysfunction in the branched chain keto-acid dehydrogenase (BCKDH) enzyme. This leads to buildup of branched-chain keto-acids (BCKA) and branched-chain amino acids (BCAA) in body fluids (e.g. keto-isocaproic acid from the BCAA le...
Autores principales: | S. Sonnet, Davis, N. O’Leary, Monique, A. Gutierrez, Mark, M. Nguyen, Steven, Mateen, Samiha, Hsu, Yuehmei, P. Mitchell, Kylie, J. Lopez, Antonio, Vockley, Jerry, K. Kennedy, Brian, Ramanathan, Arvind |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4931503/ https://www.ncbi.nlm.nih.gov/pubmed/27373929 http://dx.doi.org/10.1038/srep28775 |
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