Cross-comparison of systemic and tissue-specific metabolomes in a mouse model of Leigh syndrome

Aberrant and glutamate metabolism linked to regional neurodegeneration in a mouse model of Leigh syndrome

(2021) Karin Terburgh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

The methyltransferase METTL9 mediates pervasive 1-methylhistidine modification in mammalian proteomes

(2021) Erna Davydova et al.   Nature Communications

Stimulation of cholesterol biosynthesis in mitochondrial complex I-deficiency lowers reductive stress and improves motor function and survival in mice

(2021) Tom J.J. Schirris et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Glycerol-3-phosphate biosynthesis regenerates cytosolic NAD+ to alleviate mitochondrial disease

(2021) Shanshan Liu et al.   Cell Metabolism

Sex-related differences in bone metabolism in osteoporosis observational study

(2021) Kyu Hwan Choi et al.   MEDICINE

Roux-en-Y gastric bypass-induced bacterial perturbation contributes to altered host-bacterial co-metabolic phenotype

(2021) Jia V. Li et al.   Microbiome

Proline biosynthesis is a vent for TGFβ‐induced mitochondrial redox stress

(2020) Simon Schwörer et al.   EMBO JOURNAL

Serine Catabolism Feeds NADH when Respiration Is Impaired

(2020) Lifeng Yang et al.   Cell Metabolism

Mitochondrial disease in children

(2020) S. Rahman   JOURNAL OF INTERNAL MEDICINE

Impaired mitochondrial complex I function as a candidate driver in the biological stress response and a concomitant stress-induced brain metabolic reprogramming in male mice

(2020) Tim L. Emmerzaal et al.   Translational Psychiatry

Regional metabolic signatures in the Ndufs4(KO) mouse brain implicate defective glutamate/α-ketoglutarate metabolism in mitochondrial disease

(2020) Simon C. Johnson et al.   MOLECULAR GENETICS AND METABOLISM

Mitochondrial Proteome of Affected Glutamatergic Neurons in a Mouse Model of Leigh Syndrome

(2020) Alejandro Gella et al.   Frontiers in Cell and Developmental Biology

Metallothionein 1 Overexpression Does Not Protect Against Mitochondrial Disease Pathology in Ndufs4 Knockout Mice

(2020) Hayley Christy Miller et al.   MOLECULAR NEUROBIOLOGY

The extracellular lactate-to-pyruvate ratio modulates the sensitivity to oxidative stress-induced apoptosis via the cytosolic NADH/NAD+ redox state

(2020) Simei Go et al.   APOPTOSIS

Targeting NAD+ Metabolism as Interventions for Mitochondrial Disease

(2019) Chi Fung Lee et al.   Scientific Reports

Metabolite Exchange between Mammalian Organs Quantified in Pigs

(2019) Cholsoon Jang et al.   Cell Metabolism

Meta-omics analysis of elite athletes identifies a performance-enhancing microbe that functions via lactate metabolism

(2019) Jonathan Scheiman et al.   NATURE MEDICINE

Host mitochondria influence gut microbiome diversity: A role for ROS

(2019) Tal Yardeni et al.   Science Signaling

Defined neuronal populations drive fatal phenotype in a mouse model of Leigh syndrome

(2019) Irene Bolea et al.   eLife

Rewiring of Glutamine Metabolism Is a Bioenergetic Adaptation of Human Cells with Mitochondrial DNA Mutations

(2018) Qiuying Chen et al.   Cell Metabolism

Sex differences in metabolism and cardiometabolic disorders

(2018) Karthickeyan Chella Krishnan et al.   CURRENT OPINION IN LIPIDOLOGY

Oxygen in mitochondrial disease: can there be too much of a good thing?

(2018) Vamsi K. Mootha et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Mitochondrial medicine in the omics era

(2018) Joyeeta Rahman et al.   LANCET

A urinary biosignature for mitochondrial myopathy, encephalopathy, lactic acidosis and stroke like episodes (MELAS)

(2018) Karien Esterhuizen et al.   MITOCHONDRION

MetaboAnalyst 4.0: towards more transparent and integrative metabolomics analysis

(2018) Jasmine Chong et al.   NUCLEIC ACIDS RESEARCH

Branched-chain amino acids in health and disease: metabolism, alterations in blood plasma, and as supplements

(2018) Milan Holeček   Nutrition & Metabolism

Targeted metabolomic profiling in rat tissues reveals sex differences

(2018) Margherita Ruoppolo et al.   Scientific Reports

An integrated view of sex differences in metabolic physiology and disease

(2018) Deborah J. Clegg et al.   Molecular Metabolism

Sex differences in lipid metabolism are affected by presence of the gut microbiota

(2018) Annemarie Baars et al.   Scientific Reports

Metabolomics of Ndufs4−/− skeletal muscle: Adaptive mechanisms converge at the ubiquinone-cycle

(2018) Karin Terburgh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

SETD3 is an actin histidine methyltransferase that prevents primary dystocia

(2018) Alex W. Wilkinson et al.   NATURE

AAV9-based gene therapy partially ameliorates the clinical phenotype of a mouse model of Leigh syndrome

(2017) I Di Meo et al.   GENE THERAPY

Age and Sex Effects on Plasma Metabolite Association Networks in Healthy Subjects

(2017) Alessia Vignoli et al.   JOURNAL OF PROTEOME RESEARCH

Metabolomics of mitochondrial disease

(2017) Karien Esterhuizen et al.   MITOCHONDRION

Hepatic S6K1 Partially Regulates Lifespan of Mice with Mitochondrial Complex I Deficiency

(2017) Takashi K. Ito et al.   Frontiers in Genetics

Mitochondrial complex I-linked disease

(2016) Richard J. Rodenburg   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism

(2016) Joni Nikkanen et al.   Cell Metabolism

A charge-suppressing strategy for probing protein methylation

(2016) Zhibin Ning et al.   CHEMICAL COMMUNICATIONS

Hypoxia as a therapy for mitochondrial disease

(2016) I. H. Jain et al.   SCIENCE

Mitochondrial dysfunction remodels one-carbon metabolism in human cells

(2016) Xiaoyan Robert Bao et al.   eLife

Ethanol Production by Selected Intestinal Microorganisms and Lactic Acid Bacteria Growing under Different Nutritional Conditions

(2016) Fouad M. F. Elshaghabee et al.   Frontiers in Microbiology

Leigh syndrome: One disorder, more than 75 monogenic causes

(2015) Nicole J. Lake et al.   ANNALS OF NEUROLOGY

Skeletal muscle mitochondria of NDUFS4−/− mice display normal maximal pyruvate oxidation and ATP production

(2015) Mohammad T. Alam et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Glial Lipid Droplets and ROS Induced by Mitochondrial Defects Promote Neurodegeneration

(2015) Lucy Liu et al.   CELL

Acetyl Coenzyme A: A Central Metabolite and Second Messenger

(2015) Federico Pietrocola et al.   Cell Metabolism

Physiological conditions can be reflected in human urine proteome and metabolome

(2015) Jianqiang Wu et al.   Expert Review of Proteomics

Succination is Increased on Select Proteins in the Brainstem of the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) Knockout Mouse, a Model of Leigh Syndrome

(2015) Gerardo G. Piroli et al.   MOLECULAR & CELLULAR PROTEOMICS

The Arachidonic Acid Metabolome Serves as a Conserved Regulator of Cholesterol Metabolism

(2014) Egon Demetz et al.   Cell Metabolism

Mitochondrial Complex I Activity Suppresses Inflammation and Enhances Bone Resorption by Shifting Macrophage-Osteoclast Polarization

(2014) Zixue Jin et al.   Cell Metabolism

Identifying Small Molecules via High Resolution Mass Spectrometry: Communicating Confidence

(2014) Emma L. Schymanski et al.   ENVIRONMENTAL SCIENCE & TECHNOLOGY

Metabolite transfer with the fermentation product 2,3-butanediol enhances virulence by Pseudomonas aeruginosa

(2014) Arvind Venkataraman et al.   ISME Journal

Glutamine Oxidation Maintains the TCA Cycle and Cell Survival during Impaired Mitochondrial Pyruvate Transport

(2014) Chendong Yang et al.   MOLECULAR CELL

Complex I Deficiency Due to Selective Loss of Ndufs4 in the Mouse Heart Results in Severe Hypertrophic Cardiomyopathy

(2014) Edward T. Chouchani et al.   PLoS One

Oxidation of Alpha-Ketoglutarate Is Required for Reductive Carboxylation in Cancer Cells with Mitochondrial Defects

(2014) Andrew R. Mullen et al.   Cell Reports

Gender Differences in Skeletal Muscle Substrate Metabolism – Molecular Mechanisms and Insulin Sensitivity

(2014) Anne-Marie Lundsgaard et al.   Frontiers in Endocrinology

Gut Microbiota Affects Sensitivity to Acute DSS-induced Colitis Independently of Host Genotype

(2013) Brigitta M. Brinkman et al.   INFLAMMATORY BOWEL DISEASES

mTOR Inhibition Alleviates Mitochondrial Disease in a Mouse Model of Leigh Syndrome

(2013) S. C. Johnson et al.   SCIENCE

Interplay between Lipids and Branched-Chain Amino Acids in Development of Insulin Resistance

(2012) Christopher B. Newgard   Cell Metabolism

The Cycling of Acetyl-Coenzyme A Through Acetylcarnitine Buffers Cardiac Substrate Supply

(2012) Marie A. Schroeder et al.   Circulation-Cardiovascular Imaging

Deletion of Betaine-HomocysteineS-Methyltransferase in Mice Perturbs Choline and 1-Carbon Metabolism, Resulting in Fatty Liver and Hepatocellular Carcinomas

(2011) Ya-Wen Teng et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Fiber Types in Mammalian Skeletal Muscles

(2011) Stefano Schiaffino et al.   PHYSIOLOGICAL REVIEWS

Proline and hydroxyproline metabolism: implications for animal and human nutrition

(2010) Guoyao Wu et al.   AMINO ACIDS

Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome

(2010) A. Quintana et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Malonyl-CoA, a Key Signaling Molecule in Mammalian Cells

(2008) David Saggerson   Annual Review of Nutrition

Mice with Mitochondrial Complex I Deficiency Develop a Fatal Encephalomyopathy

(2008) Shane E. Kruse et al.   Cell Metabolism

Betaine-HomocysteineS-Methyltransferase-2 Is anS-Methylmethionine-Homocysteine Methyltransferase

(2008) Sandra S. Szegedi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

High-throughput tissue extraction protocol for NMR- and MS-based metabolomics

(2007) Huifeng Wu et al.   ANALYTICAL BIOCHEMISTRY