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

Molecular basis of the Keap1–Nrf2 system

(2015) Takafumi Suzuki et al.   FREE RADICAL BIOLOGY AND MEDICINE

Effects of metformin and other biguanides on oxidative phosphorylation in mitochondria

(2014) Hannah R. Bridges et al.   BIOCHEMICAL JOURNAL

The 2-Oxoacid Dehydrogenase Complexes in Mitochondria Can Produce Superoxide/Hydrogen Peroxide at Much Higher Rates Than Complex I

(2014) Casey L. Quinlan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Metformin suppresses gluconeogenesis by inhibiting mitochondrial glycerophosphate dehydrogenase

(2014) Anila K. Madiraju et al.   NATURE

The Target of Metformin in Type 2 Diabetes

(2014) Ele Ferrannini   NEW ENGLAND JOURNAL OF MEDICINE

The protease Omi regulates mitochondrial biogenesis through the GSK3β/PGC-1α pathway

(2014) R Xu et al.   Cell Death & Disease

Amino acid signalling upstream of mTOR

(2013) Jenna L. Jewell et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

A Novel Regulatory Defect in the Branched-Chain α-Keto Acid Dehydrogenase Complex Due to a Mutation in thePPM1KGene Causes a Mild Variant Phenotype of Maple Syrup Urine Disease

(2012) Alfonso Oyarzabal et al.   HUMAN MUTATION

The Human Serum Metabolome

(2011) Nikolaos Psychogios et al.   PLoS One

α-Ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain

(2010) Alexandre Umpierrez Amaral et al.   BRAIN RESEARCH

Abnormalities in the tricarboxylic acid (TCA) cycle in the brains of schizophrenia patients

(2010) P. Bubber et al.   EUROPEAN NEUROPSYCHOPHARMACOLOGY

Phenylbutyrate therapy for maple syrup urine disease

(2010) Nicola Brunetti-Pierri et al.   HUMAN MOLECULAR GENETICS

Metformin inhibits hepatic gluconeogenesis in mice independently of the LKB1/AMPK pathway via a decrease in hepatic energy state

(2010) Marc Foretz et al.   JOURNAL OF CLINICAL INVESTIGATION

Cause and consequence: Mitochondrial dysfunction initiates and propagates neuronal dysfunction, neuronal death and behavioral abnormalities in age-associated neurodegenerative diseases

(2009) Gary E. Gibson et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease

(2009) W. J. Zinnanti et al.   BRAIN

Protein phosphatase 2Cm is a critical regulator of branched-chain amino acid catabolism in mice and cultured cells

(2009) Gang Lu et al.   JOURNAL OF CLINICAL INVESTIGATION

Animal models of maple syrup urine disease

(2009) K. J. Skvorak   JOURNAL OF INHERITED METABOLIC DISEASE

Hepatocyte Transplantation Improves Phenotype and Extends Survival in a Murine Model of Intermediate Maple Syrup Urine Disease

(2009) Kristen J Skvorak et al.   MOLECULAR THERAPY