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...

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Publicado no:Sci Rep
Main Authors: 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
Formato: Artigo
Idioma:Inglês
Publicado em: Nature Publishing Group 2016
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4931503/
https://ncbi.nlm.nih.gov/pubmed/27373929
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep28775
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