Glutamine metabolism in diseases associated with mitochondrial dysfunction

Leukocytes mediate disease pathogenesis in the Ndufs4(KO) mouse model of Leigh syndrome

(2022) Julia C. Stokes et al.   JCI Insight

NDUFAF6-Related Leigh Syndrome Caused by Rare Pathogenic Variants: A Case Report and the Focused Review of Literature

(2022) Jaewon Kim et al.   Frontiers in Pediatrics

Impact of environmental toxicants exposure on gut-brain axis in Parkinson disease

(2022) Taiwo G. Olubodun-Obadun et al.   Drug Metabolism and Personalized Therapy

Rapamycin rescues mitochondrial dysfunction in cells carrying the m.8344A > G mutation in the mitochondrial tRNALys

(2022) Mariantonietta Capristo et al.   MOLECULAR MEDICINE

Multiple sclerosis diagnosis and phenotype identification by multivariate classification of in vivo frontal cortex metabolite profiles

(2022) Kelley M. Swanberg et al.   Scientific Reports

High-throughput sequencing analysis of nuclear-encoded mitochondrial genes reveals a genetic signature of human longevity

(2022) Brenda Gonzalez et al.   GeroScience

Glutamine Metabolism Mediates Sensitivity to Respiratory Complex II Inhibition in Acute Myeloid Leukemia

(2022) Alessia Roma et al.   MOLECULAR CANCER RESEARCH

The immune system as a driver of mitochondrial disease pathogenesis: a review of evidence

(2022) Allison Hanaford et al.   Orphanet Journal of Rare Diseases

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

Hypoglycemia in mitochondrial disorders

(2021) Myla Ashfaq et al.   MITOCHONDRION

Mitochondrial Syndromes Revisited

(2021) Daniele Orsucci et al.   Journal of Clinical Medicine

Glucose Metabolic Dysfunction in Neurodegenerative Diseases—New Mechanistic Insights and the Potential of Hypoxia as a Prospective Therapy Targeting Metabolic Reprogramming

(2021) Rongrong Han et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

The Biology and Pathobiology of Glutamatergic, Cholinergic, and Dopaminergic Signaling in the Aging Brain

(2021) Anna Gasiorowska et al.   Frontiers in Aging Neuroscience

Mechanisms underlying neonate specific metabolic effects of volatile anesthetics

(2021) Julia Stokes et al.   eLife

Common and rare variant association analyses in amyotrophic lateral sclerosis identify 15 risk loci with distinct genetic architectures and neuron-specific biology

(2021) Wouter van Rheenen et al.   NATURE GENETICS

Differential effects of mTOR inhibition and dietary ketosis in a mouse model of subacute necrotizing encephalomyelopathy

(2021) Rebecca Bornstein et al.   NEUROBIOLOGY OF DISEASE

Altered Plasma Metabolic Profiles in Chinese Patients With Multiple Sclerosis

(2021) Fan Yang et al.   Frontiers in Immunology

Alzheimer’s disease: targeting the glutamatergic system

(2020) Myra E. Conway   BIOGERONTOLOGY

Lipid-droplet-accumulating microglia represent a dysfunctional and proinflammatory state in the aging brain

(2020) Julia Marschallinger et al.   NATURE NEUROSCIENCE

Chronic Systemic Exposure to Low-Dose Rotenone Induced Central and Peripheral Neuropathology and Motor Deficits in Mice: Reproducible Animal Model of Parkinson’s Disease

(2020) Ikuko Miyazaki et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Iron, ketone bodies, and brain development

(2020) Osamu Arisaka et al.   JOURNAL OF PEDIATRICS

NAD+ Regeneration Rescues Lifespan, but Not Ataxia, in a Mouse Model of Brain Mitochondrial Complex I Dysfunction

(2020) Gregory S. McElroy et al.   Cell Metabolism

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

Lipid Droplets in Neurodegenerative Disorders

(2020) Brandon C. Farmer et al.   Frontiers in Neuroscience

Mitochondrial pathways in human health and aging

(2020) Rebecca Bornstein et al.   MITOCHONDRION

Transport of Amino Acids Across the Blood-Brain Barrier

(2020) Rosa Zaragozá   Frontiers in Physiology

Neuronal metabolic rewiring promotes resilience to neurodegeneration caused by mitochondrial dysfunction

(2020) E. Motori et al.   Science Advances

Take Advantage of Glutamine Anaplerosis, the Kernel of the Metabolic Rewiring in Malignant Gliomas

(2020) Filipa Martins et al.   Biomolecules

Effects of Ketone Bodies on Brain Metabolism and Function in Neurodegenerative Diseases

(2020) Nicole Jacqueline Jensen et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Immunological measurement of aspartate/alanine aminotransferase in predicting liver fibrosis and inflammation

(2019) Hyun Jeong Kim et al.   KOREAN JOURNAL OF INTERNAL MEDICINE

The Role of NMDA Receptors in Alzheimer’s Disease

(2019) Jinping Liu et al.   Frontiers in Neuroscience

Targeting NAD+ Metabolism as Interventions for Mitochondrial Disease

(2019) Chi Fung Lee et al.   Scientific Reports

Metabolic and oncogenic adaptations to pyruvate dehydrogenase inactivation in fibroblasts

(2019) Huabo Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

mTORC1 is required for expression of LRPPRC and cytochrome c oxidase but not HIF-1α in Leigh Syndrome French Canadian Type Patient Fibroblasts

(2019) Yvette Mukaneza et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Lipid Metabolism Crosstalk in the Brain: Glia and Neurons

(2019) Casey N. Barber et al.   Frontiers in Cellular Neuroscience

Clinical Significance of Serum Glutamine Level in Patients with Colorectal Cancer

(2019) Hang Huong Ling et al.   Nutrients

Meta-Analysis of Randomized Controlled Trials on the Efficacy and Safety of Donepezil, Galantamine, Rivastigmine, and Memantine for the Treatment of Alzheimer’s Disease

(2019) Dan-Dan Li et al.   Frontiers in Neuroscience

The Metabolomic Signature of Opa1 Deficiency in Rat Primary Cortical Neurons Shows Aspartate/Glutamate Depletion and Phospholipids Remodeling

(2019) Juan Manuel Chao de la Barca et al.   Scientific Reports

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

(2019) Irene Bolea et al.   eLife

Glutamine antagonism attenuates physical and cognitive deficits in a model of MS

(2019) Kristen R. Hollinger et al.   Neurology-Neuroimmunology & Neuroinflammation

Quantifying the Metabolic Signature of Multiple Sclerosis by in vivo Proton Magnetic Resonance Spectroscopy: Current Challenges and Future Outlook in the Translation From Proton Signal to Diagnostic Biomarker

(2019) Kelley M. Swanberg et al.   Frontiers in Neurology

Astrocytic glutamatergic transporters are involved in Aβ-induced synaptic dysfunction

(2018) Shuxuan Huang et al.   BRAIN RESEARCH

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

(2018) Qiuying Chen et al.   Cell Metabolism

Glucose Requirements of the Developing Human Brain

(2018) Manu S. Goyal et al.   JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION

Lactate in the brain: from metabolic end-product to signalling molecule

(2018) Pierre J. Magistretti et al.   NATURE REVIEWS NEUROSCIENCE

Chemotherapeutic Drugs and Mitochondrial Dysfunction: Focus on Doxorubicin, Trastuzumab, and Sunitinib

(2018) Stefania Gorini et al.   Oxidative Medicine and Cellular Longevity

Current Concepts of Neurodegenerative Mechanisms in Alzheimer’s Disease

(2018) Kasthuri Bai Magalingam et al.   Biomed Research International

NMDA antagonists for treating the non-motor symptoms in Parkinson’s disease

(2018) Brigitte Vanle et al.   Translational Psychiatry

Increased glutamate and deep brain atrophy can predict the severity of multiple sclerosis

(2018) Hubert Polacek et al.   BIOMEDICAL PAPERS-OLOMOUC

The efficacy and safety of memantine for the treatment of Alzheimer’s disease

(2018) Shinji Matsunaga et al.   Expert Opinion On Drug Safety

Paraquat exposure and Parkinson’s disease: A systematic review and meta-analysis

(2018) Wimonchat Tangamornsuksan et al.   Archives of Environmental & Occupational Health

Glutamine: Metabolism and Immune Function, Supplementation and Clinical Translation

(2018) Vinicius Cruzat et al.   Nutrients

mTOR inhibitors may benefit kidney transplant recipients with mitochondrial diseases

(2018) Simon C. Johnson et al.   KIDNEY INTERNATIONAL

The ketogenic diet as a potential treatment and prevention strategy for Alzheimer's disease

(2018) Gina M. Broom et al.   NUTRITION

Role of Glutamate and NMDA Receptors in Alzheimer’s Disease

(2017) Rui Wang et al.   JOURNAL OF ALZHEIMERS DISEASE

Mitochondrial energy generation disorders: genes, mechanisms and clues to pathology

(2017) Ann E. Frazier et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

In-vivo effects of knocking-down metabotropic glutamate receptor 5 in the SOD1 G93A mouse model of amyotrophic lateral sclerosis

(2017) Tiziana Bonifacino et al.   NEUROPHARMACOLOGY

Mitochondria in neuroinflammation – Multiple sclerosis (MS), leber hereditary optic neuropathy (LHON) and LHON-MS

(2017) David Bargiela et al.   NEUROSCIENCE LETTERS

Amino Acid Catabolism in Alzheimer’s Disease Brain: Friend or Foe?

(2017) Jeddidiah W. D. Griffin et al.   Oxidative Medicine and Cellular Longevity

Clinical effects of chemical exposures on mitochondrial function

(2017) Zarazuela Zolkipli-Cunningham et al.   TOXICOLOGY

Riluzole does not improve lifespan or motor function in three ALS mouse models

(2017) Marion C. Hogg et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Lipid Processing in the Brain: A Key Regulator of Systemic Metabolism

(2017) Kimberley D. Bruce et al.   Frontiers in Endocrinology

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

(2016) Joni Nikkanen et al.   Cell Metabolism

Network analysis of mitonuclear GWAS reveals functional networks and tissue expression profiles of disease-associated genes

(2016) Simon C. Johnson et al.   HUMAN GENETICS

Lactate as a Metabolite and a Regulator in the Central Nervous System

(2016) Patrizia Proia et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

FDG-PET study of patients with Leigh syndrome

(2016) Kauzhiro Haginoya et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse Brain

(2016) Ernst-Bernhard Kayser et al.   PLoS One

Pesticide exposure and risk of Alzheimer’s disease: a systematic review and meta-analysis

(2016) Dandan Yan et al.   Scientific Reports

Genetic evidence for common pathways in human age-related diseases

(2015) Simon C. Johnson et al.   AGING CELL

An Essential Role of the Mitochondrial Electron Transport Chain in Cell Proliferation Is to Enable Aspartate Synthesis

(2015) Kıvanç Birsoy et al.   CELL

Supporting Aspartate Biosynthesis Is an Essential Function of Respiration in Proliferating Cells

(2015) Lucas B. Sullivan et al.   CELL

The dynamic blood-brain barrier

(2015) James Keaney et al.   FEBS Journal

Energy Metabolism of the Brain, Including the Cooperation between Astrocytes and Neurons, Especially in the Context of Glycogen Metabolism

(2015) Anna Falkowska et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

PET imaging studies show enhanced expression of mGluR5 and inflammatory response during progressive degeneration in ALS mouse model expressing SOD1-G93A gene

(2015) Anna-Liisa Brownell et al.   Journal of Neuroinflammation

Progressive multiple sclerosis exhibits decreasing glutamate and glutamine over two years

(2015) EL MacMillan et al.   Multiple Sclerosis Journal

Progressive multiple sclerosis exhibits decreasing glutamate and glutamine over two years

(2015) EL MacMillan et al.   Multiple Sclerosis Journal

Neuronal glycogen synthesis contributes to physiological aging

(2014) Christopher Sinadinos et al.   AGING CELL

Roles and Regulation of Ketogenesis in Cultured Astroglia and Neurons Under Hypoxia and Hypoglycemia

(2014) Shinichi Takahashi et al.   ASN Neuro

The Glutamate Hypothesis in ALS: Pathophysiology and Drug Development

(2014) H. Blasco et al.   CURRENT MEDICINAL CHEMISTRY

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

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

Endocrine disorders in mitochondrial disease

(2013) Andrew M. Schaefer et al.   MOLECULAR AND CELLULAR ENDOCRINOLOGY

Knocking down metabotropic glutamate receptor 1 improves survival and disease progression in the SOD1G93A mouse model of amyotrophic lateral sclerosis

(2013) Marco Milanese et al.   NEUROBIOLOGY OF DISEASE

Mitochondrial-associated metabolic disorders: foundations, pathologies and recent progress

(2013) Joseph McInnes   Nutrition & Metabolism

Increased Concentrations of Glutamate and Glutamine in Normal-Appearing White Matter of Patients with Multiple Sclerosis and Normal MR Imaging Brain Scans

(2013) Anders Tisell et al.   PLoS One

Energetic cost of brain functional connectivity

(2013) D. Tomasi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2013) S. C. Johnson et al.   SCIENCE

Mitochondria as a Target of Environmental Toxicants

(2013) Joel N. Meyer et al.   TOXICOLOGICAL SCIENCES

Sugar for the brain: the role of glucose in physiological and pathological brain function

(2013) Philipp Mergenthaler et al.   TRENDS IN NEUROSCIENCES

NMDA Receptor Activity in Neuropsychiatric Disorders

(2013) Shaheen E. Lakhan et al.   Frontiers in Psychiatry

The role of mitochondrial OXPHOS dysfunction in the development of neurologic diseases

(2012) M.E. Breuer et al.   NEUROBIOLOGY OF DISEASE

The cerebellar component of Friedreich’s ataxia

(2011) Arnulf H. Koeppen et al.   ACTA NEUROPATHOLOGICA

Brain Glutamate Levels Are Decreased in Alzheimer’s Disease

(2011) Nicolas Fayed et al.   American Journal of Alzheimers Disease and Other Dementias

Glutamate NMDA receptor dysregulation in Parkinson’s disease with dyskinesias

(2011) Imtiaz Ahmed et al.   BRAIN

Rotenone and Paraquat Linked to Parkinson’s Disease: Human Exposure Study Supports Years of Animal Studies

(2011) Angela Spivey   ENVIRONMENTAL HEALTH PERSPECTIVES

Rotenone, Paraquat, and Parkinson’s Disease

(2011) Caroline M. Tanner et al.   ENVIRONMENTAL HEALTH PERSPECTIVES

Obligate Role for Ketone Body Oxidation in Neonatal Metabolic Homeostasis

(2011) David G. Cotter et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Lactate peak on brain MRS in children with syndromic mitochondrial diseases

(2011) Ching-Shiang Chi et al.   Journal of the Chinese Medical Association

Hypoxia promotes isocitrate dehydrogenase-dependent carboxylation of  -ketoglutarate to citrate to support cell growth and viability

(2011) D. R. Wise et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Late-onset progressive visual loss in a man with unusual MRI findings: MS, Harding’s, Leber’s or Leber’s Plus ?

(2010) N. Cawley et al.   IRISH JOURNAL OF MEDICAL SCIENCE

Role of Excitatory Amino Acid Transporter-2 (EAAT2) and glutamate in neurodegeneration: Opportunities for developing novel therapeutics

(2010) Keetae Kim et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Glutamate transporter variants reduce glutamate uptake in Alzheimer's disease

(2010) Heather A. Scott et al.   NEUROBIOLOGY OF AGING

A metabolomic study of the CRND8 transgenic mouse model of Alzheimer's disease

(2010) Reza M. Salek et al.   NEUROCHEMISTRY INTERNATIONAL

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

Update on the use of memantine in Alzheimer’s disease

(2010) Rob Marum   Neuropsychiatric Disease and Treatment

In vitroneurotoxic properties and excitatory aminoacids concentration in the cerebrospinal fluid of amyotrophic lateral sclerosis patients. Relationship with the degree of certainty of disease diagnoses

(2009) M. L. Fiszman et al.   ACTA NEUROLOGICA SCANDINAVICA

The blood-brain barrier and glutamate

(2009) Richard A Hawkins   AMERICAN JOURNAL OF CLINICAL NUTRITION

Cerebral oxygen and glucose metabolism in patients with mitochondrial m.3243A>G mutation

(2009) Markus M. Lindroos et al.   BRAIN

Reduced hippocampal glutamate in Alzheimer disease

(2009) R. Rupsingh et al.   NEUROBIOLOGY OF AGING

Differential Contribution of the Mitochondrial Respiratory Chain Complexes to Reactive Oxygen Species Production by Redox Cycling Agents Implicated in Parkinsonism

(2009) Derek A. Drechsel et al.   TOXICOLOGICAL SCIENCES

Tightly coupled brain activity and cerebral ATP metabolic rate

(2008) F. Du et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA