Role of Oxidative Stress in the Pathogenesis of Amyotrophic Lateral Sclerosis: Antioxidant Metalloenzymes and Therapeutic Strategies

Mutations in Superoxide Dismutase 1 (Sod1) Linked to Familial Amyotrophic Lateral Sclerosis Can Disrupt High-Affinity Zinc-Binding Promoted by the Copper Chaperone for Sod1 (Ccs)

(2020) Stefanie D. Boyd et al.   MOLECULES

Phase 1–2 Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS

(2020) Timothy Miller et al.   NEW ENGLAND JOURNAL OF MEDICINE

Trial of Sodium Phenylbutyrate–Taurursodiol for Amyotrophic Lateral Sclerosis

(2020) Sabrina Paganoni et al.   NEW ENGLAND JOURNAL OF MEDICINE

TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response

(2019) Peng Wang et al.   PLoS Genetics

Role of Catalase in Oxidative Stress- and Age-Associated Degenerative Diseases

(2019) Ankita Nandi et al.   Oxidative Medicine and Cellular Longevity

ALS Genetics, Mechanisms, and Therapeutics: Where Are We Now?

(2019) Rita Mejzini et al.   Frontiers in Neuroscience

Respiratory Failure in Amyotrophic Lateral Sclerosis

(2018) Shannon Niedermeyer et al.   CHEST

Nrf2 inhibits oxaliplatin-induced peripheral neuropathy via protection of mitochondrial function

(2018) Yang Yang et al.   FREE RADICAL BIOLOGY AND MEDICINE

Sulforaphane Inhibits the Generation of Amyloid-β Oligomer and Promotes Spatial Learning and Memory in Alzheimer’s Disease (PS1V97L) Transgenic Mice

(2018) Ting-Ting Hou et al.   JOURNAL OF ALZHEIMERS DISEASE

Sulforaphane attenuates microglia-mediated neuronal necroptosis through down-regulation of MAPK/NF-κB signaling pathways in LPS-activated BV-2 microglia

(2018) Sisi Qin et al.   PHARMACOLOGICAL RESEARCH

The secretory phenotype of senescent astrocytes isolated from Wistar newborn rats changes with anti-inflammatory drugs, but does not have a short-term effect on neuronal mitochondrial potential

(2018) Luis Ángel Maciel-Barón et al.   BIOGERONTOLOGY

Mutant FUS causes DNA ligation defects to inhibit oxidative damage repair in Amyotrophic Lateral Sclerosis

(2018) Haibo Wang et al.   Nature Communications

CuATSM Protects Against the In Vitro Cytotoxicity of Wild-Type-Like Copper–Zinc Superoxide Dismutase Mutants but not Mutants That Disrupt Metal Binding

(2018) Natalie E. Farrawell et al.   ACS Chemical Neuroscience

The yeast copper chaperone for copper-zinc superoxide dismutase (CCS1) is a multifunctional chaperone promoting all levels of SOD1 maturation

(2018) Stefanie D. Boyd et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Copper-zinc superoxide dismutase is activated through a sulfenic acid intermediate at a copper ion entry site

(2017) Morgan M. Fetherolf et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Oxygen-dependent activation of Cu,Zn-superoxide dismutase-1

(2017) Morgan M. Fetherolf et al.   Metallomics

Oxidative stress and mitochondrial damage in the pathogenesis of ALS: New perspectives

(2017) F. Bozzo et al.   NEUROSCIENCE LETTERS

TDP-43 pathology in Alzheimer's disease, dementia with Lewy bodies and ageing

(2016) Kirsty E. McAleese et al.   BRAIN PATHOLOGY

Sulforaphane prevents quinolinic acid-induced mitochondrial dysfunction in rat striatum

(2016) Erika Rubí Luis-García et al.   JOURNAL OF BIOCHEMICAL AND MOLECULAR TOXICOLOGY

Physiological functions and pathobiology of TDP-43 and FUS/TLS proteins

(2016) Antonia Ratti et al.   JOURNAL OF NEUROCHEMISTRY

Neuroinflammation increases GABAergic tone and impairs cognitive and motor function in hyperammonemia by increasing GAT-3 membrane expression. Reversal by sulforaphane by promoting M2 polarization of microglia

(2016) Vicente Hernandez-Rabaza et al.   Journal of Neuroinflammation

Sulforaphane epigenetically enhances neuronal BDNF expression and TrkB signaling pathways

(2016) Jisung Kim et al.   MOLECULAR NUTRITION & FOOD RESEARCH

The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease

(2016) Aaron R. Haeusler et al.   NATURE REVIEWS NEUROSCIENCE

Poly(GR) in C9ORF72 -Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons

(2016) Rodrigo Lopez-Gonzalez et al.   NEURON

ROS and ROS-Mediated Cellular Signaling

(2016) Jixiang Zhang et al.   Oxidative Medicine and Cellular Longevity

Glutathione peroxidase 4 and vitamin E cooperatively prevent hepatocellular degeneration

(2016) Bradley A. Carlson et al.   Redox Biology

Neuroprotective Effect of Sulforaphane against Methylglyoxal Cytotoxicity

(2015) Cristina Angeloni et al.   CHEMICAL RESEARCH IN TOXICOLOGY

Ablation of the Ferroptosis Inhibitor Glutathione Peroxidase 4 in Neurons Results in Rapid Motor Neuron Degeneration and Paralysis

(2015) Liuji Chen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The neurotoxicity of iron, copper and manganese in Parkinson's and Wilson's diseases

(2015) Petr Dusek et al.   JOURNAL OF TRACE ELEMENTS IN MEDICINE AND BIOLOGY

Sulforaphane Ameliorates Okadaic Acid-Induced Memory Impairment in Rats by Activating the Nrf2/HO-1 Antioxidant Pathway

(2015) Subhash Dwivedi et al.   MOLECULAR NEUROBIOLOGY

Sulforaphane Ameliorates 3-Nitropropionic Acid-Induced Striatal Toxicity by Activating the Keap1-Nrf2-ARE Pathway and Inhibiting the MAPKs and NF-κB Pathways

(2015) Minhee Jang et al.   MOLECULAR NEUROBIOLOGY

Vitamin E and neurodegeneration

(2015) Lynn M. Ulatowski et al.   NEUROBIOLOGY OF DISEASE

ZnII(atsm) is protective in amyotrophic lateral sclerosis model mice via a copper delivery mechanism

(2015) Erin J. McAllum et al.   NEUROBIOLOGY OF DISEASE

An acetylation switch controls TDP-43 function and aggregation propensity

(2015) Todd J. Cohen et al.   Nature Communications

Regulation of Ferroptotic Cancer Cell Death by GPX4

(2014) Wan Seok Yang et al.   CELL

The Transcription Factor Nrf2 Is a Therapeutic Target against Brain Inflammation

(2014) N. G. Innamorato et al.   JOURNAL OF IMMUNOLOGY

Oral Treatment with CuII(atsm) Increases Mutant SOD1 In Vivo but Protects Motor Neurons and Improves the Phenotype of a Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

(2014) B. R. Roberts et al.   JOURNAL OF NEUROSCIENCE

Identical twins with the C9orf72 repeat expansion are discordant for ALS

(2014) Z. Xi et al.   NEUROLOGY

In-cell NMR reveals potential precursor of toxic species from SOD1 fALS mutants

(2014) Enrico Luchinat et al.   Nature Communications

RNA-Processing Protein TDP-43 Regulates FOXO-Dependent Protein Quality Control in Stress Response

(2014) Tao Zhang et al.   PLoS Genetics

Pharmacological inhibition of cystine–glutamate exchange induces endoplasmic reticulum stress and ferroptosis

(2014) Scott J Dixon et al.   eLife

Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood

(2013) Veronique V. Belzil et al.   ACTA NEUROPATHOLOGICA

ROS Are Required for Mouse Spermatogonial Stem Cell Self-Renewal

(2013) Hiroko Morimoto et al.   Cell Stem Cell

Clinical perspective on oxidative stress in sporadic amyotrophic lateral sclerosis

(2013) Emanuele D’Amico et al.   FREE RADICAL BIOLOGY AND MEDICINE

Genistein attenuates ischemic oxidative damage and behavioral deficits via eNOS/Nrf2/HO-1 signaling

(2013) Ruimin Wang et al.   HIPPOCAMPUS

ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules

(2013) Caroline Vance et al.   HUMAN MOLECULAR GENETICS

Stress granules as crucibles of ALS pathogenesis

(2013) Yun R. Li et al.   JOURNAL OF CELL BIOLOGY

The modest impact of transcription factor Nrf2 on the course of disease in an ALS animal model

(2013) Yansu Guo et al.   LABORATORY INVESTIGATION

Dysregulation of intracellular copper homeostasis is common to transgenic mice expressing human mutant superoxide dismutase-1s regardless of their copper-binding abilities

(2013) Eiichi Tokuda et al.   NEUROBIOLOGY OF DISEASE

Global Epidemiology of Amyotrophic Lateral Sclerosis: A Systematic Review of the Published Literature

(2013) A. Chiò et al.   NEUROEPIDEMIOLOGY

Prediagnostic body fat and risk of death from amyotrophic lateral sclerosis: The EPIC cohort

(2013) V. Gallo et al.   NEUROLOGY

SOD1 and DJ-1 Converge at Nrf2 Pathway: A Clue for Antioxidant Therapeutic Potential in Neurodegeneration

(2013) Pamela Milani et al.   Oxidative Medicine and Cellular Longevity

Mitochondrial Reactive Oxygen Species Promote Epidermal Differentiation and Hair Follicle Development

(2013) R. B. Hamanaka et al.   Science Signaling

Nanoparticle-mediated catalase delivery protects human neurons from oxidative stress

(2013) A Singhal et al.   Cell Death & Disease

Therapeutic effects of CuII(atsm) in the SOD1-G37R mouse model of amyotrophic lateral sclerosis

(2013) Erin J. McAllum et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Effect of Nrf2 activators on release of glutathione, cysteinylglycine and homocysteine by human U373 astroglial cells

(2013) Megan L. Steele et al.   Redox Biology

Carnosic Acid Prevents 6-Hydroxydopamine-Induced Cell Death in SH-SY5Y Cells via Mediation of Glutathione Synthesis

(2012) Jing-Hsien Chen et al.   CHEMICAL RESEARCH IN TOXICOLOGY

Pesticide exposure as a risk factor for amyotrophic lateral sclerosis: A meta-analysis of epidemiological studies

(2012) Angela M. Malek et al.   ENVIRONMENTAL RESEARCH

De novo FUS gene mutations are associated with juvenile-onset sporadic amyotrophic lateral sclerosis in China

(2012) Zhang-Yu Zou et al.   NEUROBIOLOGY OF AGING

Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates

(2012) Sarah J. Parker et al.   NEUROCHEMISTRY INTERNATIONAL

Metals, oxidative stress and neurodegeneration: A focus on iron, manganese and mercury

(2012) Marcelo Farina et al.   NEUROCHEMISTRY INTERNATIONAL

Pesticide exposure and amyotrophic lateral sclerosis

(2012) Freya Kamel et al.   NEUROTOXICOLOGY

Iron Accumulation in Deep Cortical Layers Accounts for MRI Signal Abnormalities in ALS: Correlating 7 Tesla MRI and Pathology

(2012) Justin Y. Kwan et al.   PLoS One

Modulation of Experimental Herpes Encephalitis-Associated Neurotoxicity through Sulforaphane Treatment

(2012) Scott J. Schachtele et al.   PLoS One

Human superoxide dismutase 1 (hSOD1) maturation through interaction with human copper chaperone for SOD1 (hCCS)

(2012) L. Banci et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Reactive oxygen species: from health to disease

(2012) K Brieger et al.   SWISS MEDICAL WEEKLY

Dysregulation of Glutathione Homeostasis in Neurodegenerative Diseases

(2012) William M. Johnson et al.   Nutrients

Premorbid body mass index and risk of amyotrophic lateral sclerosis

(2012) Éilis J. O'Reilly et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

PGC-1α Regulates Translocated in Liposarcoma Activity: Role in Oxidative Stress Gene Expression

(2011) Cristina Sánchez-Ramos et al.   ANTIOXIDANTS & REDOX SIGNALING

Pharmacological Targeting of the Transcription Factor Nrf2 at the Basal Ganglia Provides Disease Modifying Therapy for Experimental Parkinsonism

(2011) Agnieszka Jazwa et al.   ANTIOXIDANTS & REDOX SIGNALING

FOXO3a regulates reactive oxygen metabolism by inhibiting mitochondrial gene expression

(2011) E C Ferber et al.   CELL DEATH AND DIFFERENTIATION

Mitochondrial Complex III ROS Regulate Adipocyte Differentiation

(2011) Kathryn V. Tormos et al.   Cell Metabolism

Nrf2 and NF-κB Modulation by Sulforaphane Counteracts Multiple Manifestations of Diabetic Neuropathy in Rats and High Glucose-Induced Changes

(2011) Geeta Negi et al.   CURRENT NEUROVASCULAR RESEARCH

Neuroprotective effect of Nrf2/ARE activators, CDDO ethylamide and CDDO trifluoroethylamide, in a mouse model of amyotrophic lateral sclerosis

(2011) Arie Neymotin et al.   FREE RADICAL BIOLOGY AND MEDICINE

Activation of stress signaling pathways by electrophilic oxidized and nitrated lipids

(2011) Emilia Kansanen et al.   FREE RADICAL BIOLOGY AND MEDICINE

Diacetylbis(N(4)-methylthiosemicarbazonato) Copper(II) (CuII(atsm)) Protects against Peroxynitrite-induced Nitrosative Damage and Prolongs Survival in Amyotrophic Lateral Sclerosis Mouse Model

(2011) Cynthia P. W. Soon et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Population based epidemiology of amyotrophic lateral sclerosis using capture-recapture methodology

(2011) M. H. B. Huisman et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Glutathione Biosynthesis via Activation of the Nuclear Factor E2–Related Factor 2 (Nrf2) – Antioxidant-Response Element (ARE) Pathway Is Essential for Neuroprotective Effects of Sulforaphane and 6-(Methylsulfinyl) Hexyl Isothiocyanate

(2011) Keita Mizuno et al.   JOURNAL OF PHARMACOLOGICAL SCIENCES

A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study

(2011) Ilse Gijselinck et al.   LANCET NEUROLOGY

Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies

(2011) Yohei Iguchi et al.   NEUROBIOLOGY OF DISEASE

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

The role of Nrf2 signaling in the regulation of antioxidants and detoxifying enzymes after traumatic brain injury in rats and mice

(2010) Yuan Hong et al.   ACTA PHARMACOLOGICA SINICA

Folding Catalysis by Transient Coordination of Zn2+to the Cu Ligands of the ALS-Associated Enzyme Cu/Zn Superoxide Dismutase 1

(2010) Lina Leinartaitė et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

(2010) Ian RA Mackenzie et al.   LANCET NEUROLOGY

Energy metabolism in amyotrophic lateral sclerosis

(2010) Luc Dupuis et al.   LANCET NEUROLOGY

Reactive oxygen species and antioxidant machinery in abiotic stress tolerance in crop plants

(2010) Sarvajeet Singh Gill et al.   PLANT PHYSIOLOGY AND BIOCHEMISTRY

ALS in Italian professional soccer players: The risk is still present and could be soccer-specific

(2009) Adriano Chiò et al.   Amyotrophic Lateral Sclerosis

What we truly know about occupation as a risk factor for ALS: A critical and systematic review

(2009) Nadia A. Sutedja et al.   Amyotrophic Lateral Sclerosis

Smoking and risk for amyotrophic lateral sclerosis: Analysis of the EPIC cohort

(2009) Valentina Gallo et al.   ANNALS OF NEUROLOGY

NOX Enzymes in the Central Nervous System: From Signaling to Disease

(2009) Silvia Sorce et al.   ANTIOXIDANTS & REDOX SIGNALING

TDP-43 Redistribution is an Early Event in Sporadic Amyotrophic Lateral Sclerosis

(2009) Maria Teresa Giordana et al.   BRAIN PATHOLOGY

Workplace Exposures and the Risk of Amyotrophic Lateral Sclerosis

(2009) Fang Fang et al.   ENVIRONMENTAL HEALTH PERSPECTIVES

Glutathione peroxidase 4 differentially regulates the release of apoptogenic proteins from mitochondria

(2009) Hanyu Liang et al.   FREE RADICAL BIOLOGY AND MEDICINE

The Right to Choose: Multiple Pathways for Activating Copper,Zinc Superoxide Dismutase

(2009) Jeffry M. Leitch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Dysregulation of intracellular copper trafficking pathway in a mouse model of mutant copper/zinc superoxide dismutase-linked familial amyotrophic lateral sclerosis

(2009) Eiichi Tokuda et al.   JOURNAL OF NEUROCHEMISTRY

Incidence of amyotrophic lateral sclerosis in Europe

(2009) G. Logroscino et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Lead exposure stimulates VEGF expression in the spinal cord and extends survival in a mouse model of ALS

(2009) Ana G. Barbeito et al.   NEUROBIOLOGY OF DISEASE

Amyotrophic lateral sclerosis

(2009) Lokesh C Wijesekera et al.   Orphanet Journal of Rare Diseases

Neuroprotective Effects of the Triterpenoid, CDDO Methyl Amide, a Potent Inducer of Nrf2-Mediated Transcription

(2009) Lichuan Yang et al.   PLoS One

The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis

(2008) Michael J. Strong   Amyotrophic Lateral Sclerosis

Nuclear Erythroid 2-Related Factor 2-Antioxidative Response Element Signaling Pathway in Motor Cortex and Spinal Cord in Amyotrophic Lateral Sclerosis

(2008) Alexander Sarlette et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Nrf2 Activation in Astrocytes Protects against Neurodegeneration in Mouse Models of Familial Amyotrophic Lateral Sclerosis

(2008) M. R. Vargas et al.   JOURNAL OF NEUROSCIENCE

Role of protein kinase Cδ in paraquat‐induced glial cell death

(2008) Sangseop Kim et al.   JOURNAL OF NEUROSCIENCE RESEARCH

TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis

(2008) Vivianna M Van Deerlin et al.   LANCET NEUROLOGY

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS

Dual roles of Nrf2 in cancer

(2008) A LAU et al.   PHARMACOLOGICAL RESEARCH

Mitochondrial complex I inhibition is not required for dopaminergic neuron death induced by rotenone, MPP+, or paraquat

(2008) W.-S. Choi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Hyperinflammation in chronic granulomatous disease and anti-inflammatory role of the phagocyte NADPH oxidase

(2008) Michela G. Schäppi et al.   Seminars in Immunopathology

Novel Mutations in TARDBP (TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis

(2008) Nicola J. Rutherford et al.   PLoS Genetics

Xenobiotic metabolizing enzymes in the central nervous system: Contribution of cytochrome P450 enzymes in normal and pathological human brain

(2007) Fabien Dutheil et al.   BIOCHIMIE

Free radicals and muscle fatigue: Of ROS, canaries, and the IOC

(2007) Michael B. Reid   FREE RADICAL BIOLOGY AND MEDICINE

Carnosic acid, acatechol-typeelectrophilic compound, protects neurons bothin vitroandin vivothrough activation of the Keap1/Nrf2 pathway viaS-alkylation of targeted cysteines on Keap1

(2007) Takumi Satoh et al.   JOURNAL OF NEUROCHEMISTRY

A neurobehavioral study of current and former welders exposed to manganese

(2007) Dag G. Ellingsen et al.   NEUROTOXICOLOGY

Biomarkers of oxidative stress and DNA damage in agricultural workers: A pilot study

(2007) Juan F. Muniz et al.   TOXICOLOGY AND APPLIED PHARMACOLOGY