4.7 Article

Neurons undergo pathogenic metabolic reprogramming in models of familial ALS

Related references

Note: Only part of the references are listed.
Article Multidisciplinary Sciences

Glycolysis fuels phosphoinositide 3-kinase signaling to bolster T cell immunity

Ke Xu et al.

Summary: The passage discusses the impact of infection on T cell immune response, highlighting the crucial role of glycolytic enzyme LDHA in regulating the PI3K-Akt-Foxo1 signaling pathway, a mechanism that serves as a key factor in controlling T cell immunity.

SCIENCE (2021)

Article Multidisciplinary Sciences

Senolysis by glutaminolysis inhibition ameliorates various age-associated disorders

Yoshikazu Johmura et al.

Summary: Research has shown that senescent cells rely on glutaminolysis for survival, and inhibiting this process can induce senolysis. Lysosomal membrane damage lowers intracellular pH in senescent cells, inducing KGA expression, leading to glutaminolysis, and ultimately producing ammonia to neutralize low pH and improve cell survival.

SCIENCE (2021)

Article Cell Biology

C9orf72 regulates energy homeostasis by stabilizing mitochondrial complex I assembly

Tao Wang et al.

Summary: Haploinsufficiency of C9orf72 is linked to ALS and FTD, with C9orf72 playing a role in regulating cellular energy balance by stabilizing TIMMDC1, a crucial component of OXPHOS complex I in mitochondria.

CELL METABOLISM (2021)

Article Neurosciences

Multi-omic analysis of selectively vulnerable motor neuron subtypes implicates altered lipid metabolism in ALS

Hojae Lee et al.

Summary: By comparing human induced pluripotent stem cell-derived spinal and ocular motor neurons, researchers identified low levels of a natural 5-lipoxygenase inhibitor in ALS spinal motor neurons and found that analogs of 5-lipoxygenase inhibitors can improve ALS phenotypes. Multi-omics analysis revealed shared metabolic perturbations in ALS spinal motor neurons, specifically dysregulation in lipid metabolism, which was confirmed through targeted metabolomics. These findings highlight a potential therapeutic target in lipid metabolism for ALS.

NATURE NEUROSCIENCE (2021)

Review Pharmacology & Pharmacy

Treatment strategies for glucose-6-phosphate dehydrogenase deficiency: past and future perspectives

Adriana A. Garcia et al.

Summary: G6PD plays a crucial role in maintaining redox balance and resistance to oxidative stress, with deficiency affecting millions worldwide and potentially leading to acute hemolytic anemia. Despite the lack of therapeutics for G6PD deficiency, discussions on potential pharmacological pathways and treatment strategies have been undertaken.

TRENDS IN PHARMACOLOGICAL SCIENCES (2021)

Article Multidisciplinary Sciences

Coupling of activity, metabolism and behaviour across the Drosophila brain

Kevin Mann et al.

Summary: The coordinated activity of neurons in networks plays a crucial role in both resting and active behavioral states across species. Changes in neural activity are shown to drive alterations in metabolic flux, with even transient increases in activity resulting in persistent changes in energy metabolism. This relationship between neural activity and energy metabolism is likely evolutionarily ancient and highly conserved, providing a foundation for using metabolic proxies to capture changes in neural activity.

NATURE (2021)

Article Multidisciplinary Sciences

TDP-43 and PINK1 mediate CHCHD10S59L mutation-induced defects in Drosophila and in vitro

Minwoo Baek et al.

Summary: Mutations in CHCHD10 can lead to ALS and FTD. The CHCHD10S59L mutation causes cell toxicity and mitochondrial defects through the TDP-43 and PINK1 pathways.

NATURE COMMUNICATIONS (2021)

Article Multidisciplinary Sciences

Neuronal mitochondrial dysfunction in sporadic amyotrophic lateral sclerosis is developmentally regulated

Tanisha Singh et al.

Summary: This study revealed a unified mitochondrial dysfunction phenotype in motor neurons of sALS and fALS patients, suggesting that treatments aimed at rescuing mitochondrial function may be beneficial for both types of ALS patients.

SCIENTIFIC REPORTS (2021)

Article Multidisciplinary Sciences

Mechanical regulation of glycolysis via cytoskeleton architecture

Jin Suk Park et al.

NATURE (2020)

Article Biochemistry & Molecular Biology

A small molecule G6PD inhibitor reveals immune dependence on pentose phosphate pathway

Jonathan M. Ghergurovich et al.

NATURE CHEMICAL BIOLOGY (2020)

Article Biochemistry & Molecular Biology

ATP Synthase c-Subunit Leak Causes Aberrant Cellular Metabolism in Fragile X Syndrome

Pawel Licznerski et al.

Article Biochemistry & Molecular Biology

TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS

Chien-Hsiung Yu et al.

Review Biochemistry & Molecular Biology

Neuroprotective Effect of Antioxidants in the Brain

Kyung Hee Lee et al.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2020)

Review Neurosciences

The role of mitochondria in amyotrophic lateral sclerosis

Emma F. Smith et al.

NEUROSCIENCE LETTERS (2019)

Review Neurosciences

Mitochondrial integrity in neurodegeneration

Katrina Cowan et al.

CNS NEUROSCIENCE & THERAPEUTICS (2019)

Review Neurosciences

Creatine as a Neuroprotector: an Actor that Can Play Many Parts

Eduardo Peil Marques et al.

NEUROTOXICITY RESEARCH (2019)

Article Neurosciences

Pleiotropic Mitochondria: The Influence of Mitochondria on Neuronal Development and Disease

Vidhya Rangaraju et al.

JOURNAL OF NEUROSCIENCE (2019)

Article Neurosciences

TDP-43 proteinopathy and mitochondrial abnormalities in neurodegeneration

Ju Gao et al.

MOLECULAR AND CELLULAR NEUROSCIENCE (2019)

Review Medicine, Research & Experimental

Mitochondria, OxPhos, and neurodegeneration: cells are not just running out of gas

Estela Area-Gomez et al.

JOURNAL OF CLINICAL INVESTIGATION (2019)

Review Physiology

BRAIN GLUCOSE METABOLISM: INTEGRATION OF ENERGETICS WITH FUNCTION

Gerald A. Dienel

PHYSIOLOGICAL REVIEWS (2019)

Article Biochemistry & Molecular Biology

Loss of CHCHD10-CHCHD2 complexes required for respiration underlies the pathogenicity of a CHCHD10 mutation in ALS

Isabella R. Straub et al.

HUMAN MOLECULAR GENETICS (2018)

Article Endocrinology & Metabolism

Evaluating the gray and white matter energy budgets of human brain function

Yuguo Yu et al.

JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM (2018)

Review Clinical Neurology

Energy metabolism in ALS: an underappreciated opportunity?

Tijs Vandoorne et al.

ACTA NEUROPATHOLOGICA (2018)

Review Biochemistry & Molecular Biology

NADPH oxidases as drug targets and biomarkers in neurodegenerative diseases: What is the evidence?

Silvia Sorce et al.

FREE RADICAL BIOLOGY AND MEDICINE (2017)

Article Cell Biology

Glycolytic Enzymes Coalesce in G Bodies under Hypoxic Stress

Meiyan Jin et al.

CELL REPORTS (2017)

Article Biochemistry & Molecular Biology

Evaluation of NADPH oxidases as drug targets in a mouse model of familial amyotrophic lateral sclerosis

Tamara Seredenina et al.

FREE RADICAL BIOLOGY AND MEDICINE (2016)

Review Oncology

Glucose-6-Phosphate Dehydrogenase Deficiency

Lucio Luzzatto et al.

HEMATOLOGY-ONCOLOGY CLINICS OF NORTH AMERICA (2016)

Article Biochemistry & Molecular Biology

The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity

Wenzhang Wang et al.

NATURE MEDICINE (2016)

Review Multidisciplinary Sciences

Fundamentals of cancer metabolism

Ralph J. DeBerardinis et al.

SCIENCE ADVANCES (2016)

Article Neurosciences

Loss of RAD-23 Protects Against Models of Motor Neuron Disease by Enhancing Mutant Protein Clearance

Angela M. Jablonski et al.

JOURNAL OF NEUROSCIENCE (2015)

Article Neurosciences

Inhibition of Cytohesins Protects against Genetic Models of Motor Neuron Disease

Jinbin Zhai et al.

JOURNAL OF NEUROSCIENCE (2015)

Article Biochemistry & Molecular Biology

Regulation of Protein Quality Control by UBE4B and LSD1 through p53-Mediated Transcription

Goran Periz et al.

PLOS BIOLOGY (2015)

Review Medicine, Research & Experimental

The Role of Oxidative Stress in Neurodegenerative Diseases

Geon Ha Kim et al.

EXPERIMENTAL NEUROBIOLOGY (2015)

Article Biochemistry & Molecular Biology

Hexosamine Pathway Metabolites Enhance Protein Quality Control and Prolong Life

Martin S. Denzel et al.

Article Biochemistry & Molecular Biology

Activity-Driven Local ATP Synthesis Is Required for Synaptic Function

Vidhya Rangaraju et al.

Article Cell & Tissue Engineering

Pathways Disrupted in Human ALS Motor Neurons Identified through Genetic Correction of Mutant SOD1

Evangelos Kiskinis et al.

CELL STEM CELL (2014)

Article Biochemistry & Molecular Biology

The Molecular and Metabolic Influence of Long Term Agmatine Consumption*

Itzhak Nissim et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2014)

Article Clinical Neurology

NADPH oxidase (NOX2) activity is a modifier of survival in ALS

Giuseppe Marrali et al.

JOURNAL OF NEUROLOGY (2014)

Article Multidisciplinary Sciences

The metabolite α-ketoglutarate extends lifespan by inhibiting ATP synthase and TOR

Randall M. Chin et al.

NATURE (2014)

Article Multidisciplinary Sciences

Fructose-1,6-bisphosphatase opposes renal carcinoma progression

Bo Li et al.

NATURE (2014)

Article Multidisciplinary Sciences

C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins

Sarah Mizielinska et al.

SCIENCE (2014)

Article Biochemical Research Methods

Structural basis of high-order oligomerization of the cullin-3 adaptor SPOP

Laura K. van Geersdaele et al.

ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY (2013)

Article Biochemistry & Molecular Biology

Vesicular Glycolysis Provides On-Board Energy for Fast Axonal Transport

Diana Zala et al.

Article Biochemical Research Methods

A new LC-MS/MS method for the clinical determination of reduced and oxidized glutathione from whole blood

Tereza Moore et al.

JOURNAL OF CHROMATOGRAPHY B-ANALYTICAL TECHNOLOGIES IN THE BIOMEDICAL AND LIFE SCIENCES (2013)

Article Biochemistry & Molecular Biology

S-nitrosylated protein disulfide isomerase contributes to mutant SOD1 aggregates in amyotrophic lateral sclerosis

Xueping Chen et al.

JOURNAL OF NEUROCHEMISTRY (2013)

Review Multidisciplinary Sciences

The nexus of chromatin regulation and intermediary metabolism

Philipp Gut et al.

NATURE (2013)

Review Medicine, General & Internal

Mitochondrial Dynamics - Mitochondrial Fission and Fusion in Human Diseases

Stephen L. Archer

NEW ENGLAND JOURNAL OF MEDICINE (2013)

Article Biochemistry & Molecular Biology

Effects of a glucokinase activator on hepatic intermediary metabolism: study with 13C-isotopomer-based metabolomics

Itzhak Nissim et al.

BIOCHEMICAL JOURNAL (2012)

Review Biochemistry & Molecular Biology

Glucose-6-phosphate dehydrogenase, NADPH, and cell survival

Robert C. Stanton

IUBMB LIFE (2012)

Article Genetics & Heredity

Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models

Maria Armakola et al.

NATURE GENETICS (2012)

Review Cell Biology

AMPK: a nutrient and energy sensor that maintains energy homeostasis

D. Grahame Hardie et al.

NATURE REVIEWS MOLECULAR CELL BIOLOGY (2012)

Review Neurosciences

Nitric Oxide-Mediated Oxidative Damage and the Progressive Demise of Motor Neurons in ALS

Derek A. Drechsel et al.

NEUROTOXICITY RESEARCH (2012)

Article Genetics & Heredity

New and Redesigned pRS Plasmid Shuttle Vectors for Genetic Manipulation of Saccharomyces cerevisiae

Mark K. Chee et al.

G3-GENES GENOMES GENETICS (2012)

Review Cell Biology

The diverse functions of GAPDH: Views from different subcellular compartments

Carlos Tristan et al.

CELLULAR SIGNALLING (2011)

Review Endocrinology & Metabolism

Response to 'comment on recent modeling studies of astrocyte-neuron metabolic interactions': much ado about nothing

Silvia Mangia et al.

JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM (2011)

Review Cell Biology

The AMPK signalling pathway coordinates cell growth, autophagy and metabolism

Maria M. Mihaylova et al.

NATURE CELL BIOLOGY (2011)

Article Multidisciplinary Sciences

A yeast functional screen predicts new candidate ALS disease genes

Julien Couthouis et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)

Review Oncology

O-GlcNAc signalling: implications for cancer cell biology

Chad Slawson et al.

NATURE REVIEWS CANCER (2011)

Editorial Material Endocrinology & Metabolism

Comment on recent modeling studies of astrocyte-neuron metabolic interactions

Renaud Jolivet et al.

JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM (2010)

Article Neurosciences

FOXO3a Is Broadly Neuroprotective In Vitro and In Vivo against Insults Implicated in Motor Neuron Diseases

Jelena Mojsilovic-Petrovic et al.

JOURNAL OF NEUROSCIENCE (2009)

Article Neurosciences

NADPH oxidase is the primary source of superoxide induced by NMDA receptor activation

Angela M. Brennan et al.

NATURE NEUROSCIENCE (2009)

Article Biochemistry & Molecular Biology

Human αB-crystallin mutation causes oxido-reductive stress and protein aggregation cardiomyopathy in mice

Namakkal S. Rajasekaran et al.

Review Biochemistry & Molecular Biology

Oxidative stress in ALS: A mechanism of neurodegeneration and a therapeutic target

Sian C. Barber et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2006)

Review Multidisciplinary Sciences

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

Michael T. Lin et al.

NATURE (2006)

Review Neurosciences

Protecting motor neurons from toxic insult by antagonism of adenosine A2a and Trk receptors

Jelena Mojsilovic-Petrovic et al.

JOURNAL OF NEUROSCIENCE (2006)

Article Multidisciplinary Sciences

Evidence for defective energy homeostasis in amyotrophic lateral sclerosis:: benefit of a high-energy diet in a transgenic mouse

L Dupuis et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2004)

Article Biochemistry & Molecular Biology

Glucose-6-phosphate dehydrogenase modulates vascular endothelial growth factor-mediated angiogenesis

JA Leopold et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2003)

Article Biochemistry & Molecular Biology

Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice

M Mattiazzi et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2002)

Article Biochemistry & Molecular Biology

Compartmentation of TCA cycle metabolism in cultured neocortical neurons revealed by 13C MR spectroscopy

HS Waagepetersen et al.

NEUROCHEMISTRY INTERNATIONAL (2000)

Article Multidisciplinary Sciences

Mitochondrial dysfunction during hypoxia/reoxygenation and its correction by anaerobic metabolism of citric acid cycle intermediates

JM Weinberg et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2000)