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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...

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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group 2016
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