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Article

Metformin inhibits Branched Chain Amino Acid (BCAA) derived ketoacidosis and promotes metabolic homeostasis in MSUD.

Scientific reports - 4 Jul 2016

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

Abstract excerpt

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 leucine), leading to numerous clinical features including a less understood skeletal muscle dysfunction in patients....

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Share to Amino Acids, Branched-Chain
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 27373929
DOI: 10.1038/srep28775
PMCID: PMC4931503

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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