Risk Factors and Emerging Therapies in Amyotrophic Lateral Sclerosis

Early post-marketing experience with edaravone in an unselected group of patients with ALS

(2019) Alon Abraham et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Edaravone for the treatment of amyotrophic lateral sclerosis

(2019) Hiide Yoshino   Expert Review of Neurotherapeutics

The molecular pathogenesis of superoxide dismutase 1-linked ALS is promoted by low oxygen tension

(2019) Isil Keskin et al.   ACTA NEUROPATHOLOGICA

Targeting RNA-Mediated Toxicity in C9orf72 ALS and/or FTD by RNAi-Based Gene Therapy

(2019) Raygene Martier et al.   Molecular Therapy-Nucleic Acids

Structural insights into the ubiquitin recognition by OPTN (optineurin) and its regulation by TBK1-mediated phosphorylation

(2018) Faxiang Li et al.   Autophagy

SOD1 in Amyotrophic Lateral Sclerosis: “Ambivalent” Behavior Connected to the Disease

(2018) Orietta Pansarasa et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Riluzole, disease stage and survival in ALS

(2018) Thanuja Dharmadasa et al.   LANCET NEUROLOGY

Stage at which riluzole treatment prolongs survival in patients with amyotrophic lateral sclerosis: a retrospective analysis of data from a dose-ranging study

(2018) Ton Fang et al.   LANCET NEUROLOGY

Amyotrophic Lateral Sclerosis: An Update for 2018

(2018) Björn Oskarsson et al.   MAYO CLINIC PROCEEDINGS

Motor neuron disease in 2017: Progress towards therapy in motor neuron disease

(2018) Matthew C. Kiernan   Nature Reviews Neurology

Association between TBK1 mutations and risk of amyotrophic lateral sclerosis/frontotemporal dementia spectrum: a meta-analysis

(2018) Rongrong Cui et al.   NEUROLOGICAL SCIENCES

MIF inhibits the formation and toxicity of misfolded SOD1 amyloid aggregates: implications for familial ALS

(2018) Neta Shvil et al.   Cell Death & Disease

“ALS reversals”: demographics, disease characteristics, treatments, and co-morbidities

(2018) Daniel Harrison et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Riluzole and edaravone: A tale of two amyotrophic lateral sclerosis drugs

(2018) Manoj Kumar Jaiswal   MEDICINAL RESEARCH REVIEWS

The link of organophosphorus pesticides with neurodegenerative and neurodevelopmental diseases based on evidence and mechanisms

(2018) Sara Mostafalou et al.   TOXICOLOGY

Aggregated SOD1 causes selective death of cultured human motor neurons

(2018) Chen Benkler et al.   Scientific Reports

Systemic injection of AAV9-GDNF provides modest functional improvements in the SOD1G93A ALS rat but has adverse side effects

(2017) G M Thomsen et al.   GENE THERAPY

A Meta-Analysis on the Relationship of the PON Genes and Alzheimer Disease

(2017) Yi Nie et al.   JOURNAL OF GERIATRIC PSYCHIATRY AND NEUROLOGY

Genetic epidemiology of amyotrophic lateral sclerosis: a systematic review and meta-analysis

(2017) Zhang-Yu Zou et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Identification of risk factors associated with onset and progression of amyotrophic lateral sclerosis using systematic review and meta-analysis

(2017) Ming-Dong Wang et al.   NEUROTOXICOLOGY

Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation

(2017) Tariq Afroz et al.   Nature Communications

ALS Clinical Trials Review: 20 Years of Failure. Are We Any Closer to Registering a New Treatment?

(2017) Dmitry Petrov et al.   Frontiers in Aging Neuroscience

Edaravone and its clinical development for amyotrophic lateral sclerosis

(2017) Koji Takei et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Disease-modifying and symptomatic treatment of amyotrophic lateral sclerosis

(2017) Johannes Dorst et al.   Therapeutic Advances in Neurological Disorders

Non-neuronal Cells in ALS: Role of Glial, Immune cells and Blood-CNS Barriers

(2016) Fabiola Puentes et al.   BRAIN PATHOLOGY

Pathogenesis of amyotrophic lateral sclerosis

(2016) Sarah Morgan et al.   BRITISH MEDICAL BULLETIN

Stem cells therapy for ALS

(2016) Letizia Mazzini et al.   EXPERT OPINION ON BIOLOGICAL THERAPY

Amyotrophic lateral sclerosis and environmental factors

(2016) Virginia Bozzoni   FUNCTIONAL NEUROLOGY

Quantification of the Relative Contributions of Loss-of-function and Gain-of-function Mechanisms in TAR DNA-binding Protein 43 (TDP-43) Proteinopathies

(2016) Roberta Cascella et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

GPNMB ameliorates mutant TDP-43-induced motor neuron cell death

(2016) Yuki Nagahara et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Impaired Autophagy and Defective Mitochondrial Function: Converging Paths on the Road to Motor Neuron Degeneration

(2016) Brittany M. Edens et al.   Frontiers in Cellular Neuroscience

Riluzole exerts transient modulating effects on cortical and axonal hyperexcitability in ALS

(2016) Nimeshan Geevasinga et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Whole-genome sequencing reveals important role for TBK1 and OPTN mutations in frontotemporal lobar degeneration without motor neuron disease

(2015) Cyril Pottier et al.   ACTA NEUROPATHOLOGICA

Tauroursodeoxycholic acid in the treatment of patients with amyotrophic lateral sclerosis

(2015) A. E. Elia et al.   EUROPEAN JOURNAL OF NEUROLOGY

Human glial progenitor engraftment and gene expression is independent of the ALS environment

(2015) Amanda M. Haidet-Phillips et al.   EXPERIMENTAL NEUROLOGY

Erythropoietin in amyotrophic lateral sclerosis: a multicentre, randomised, double blind, placebo controlled, phase III study

(2015) Giuseppe Lauria et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Oxidative Stress in Neurodegenerative Diseases

(2015) Ewa Niedzielska et al.   MOLECULAR NEUROBIOLOGY

Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia

(2015) Axel Freischmidt et al.   NATURE NEUROSCIENCE

Amyotrophic lateral sclerosis: mechanisms and therapeutics in the epigenomic era

(2015) Ximena Paez-Colasante et al.   Nature Reviews Neurology

Potential Environmental Factors in Amyotrophic Lateral Sclerosis

(2015) Björn Oskarsson et al.   NEUROLOGIC CLINICS

Transcriptomic Changes Due to Cytoplasmic TDP-43 Expression Reveal Dysregulation of Histone Transcripts and Nuclear Chromatin

(2015) Alexandre Amlie-Wolf et al.   PLoS One

Delayed Disease Onset and Extended Survival in the SOD1G93A Rat Model of Amyotrophic Lateral Sclerosis after Suppression of Mutant SOD1 in the Motor Cortex

(2014) G. M. Thomsen et al.   JOURNAL OF NEUROSCIENCE

A Meta-Analysis of Observational Studies of the Association Between Chronic Occupational Exposure to Lead and Amyotrophic Lateral Sclerosis

(2014) Ming-Dong Wang et al.   JOURNAL OF OCCUPATIONAL AND ENVIRONMENTAL MEDICINE

Advances in motor neurone disease

(2014) Dirk Bäumer et al.   JOURNAL OF THE ROYAL SOCIETY OF MEDICINE

Extracellular Vesicles from Neural Stem Cells Transfer IFN-γ via Ifngr1 to Activate Stat1 Signaling in Target Cells

(2014) Chiara Cossetti et al.   MOLECULAR CELL

7,8-Dihydroxyflavone improves motor performance and enhances lower motor neuronal survival in a mouse model of amyotrophic lateral sclerosis

(2014) Orhan Tansel Korkmaz et al.   NEUROSCIENCE LETTERS

Dexpramipexole Is Ineffective in Two Models of ALS Related Neurodegeneration

(2014) Fernando G. Vieira et al.   PLoS One

A post hoc analysis of subgroup outcomes and creatinine in the phase III clinical trial (EMPOWER) of dexpramipexole in ALS

(2014) Michael E. Bozik et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Amyotrophic Lateral Sclerosis - Cell Based Therapy and Novel Therapeutic Development

(2014) Changsung Kim et al.   Experimental Neurobiology

Weight loss, dysphagia and supplement intake in patients with amyotrophic lateral sclerosis (ALS): impact on quality of life and therapeutic options

(2013) Sonja Körner et al.   BMC Neurology

Is SOD1 loss of function involved in amyotrophic lateral sclerosis?

(2013) Rachele A. Saccon et al.   BRAIN

Mitochondrial dysfunction in amyotrophic lateral sclerosis - a valid pharmacological target?

(2013) H Muyderman et al.   BRITISH JOURNAL OF PHARMACOLOGY

Clinical perspective on oxidative stress in sporadic amyotrophic lateral sclerosis

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

Exposure to Environmental Toxicants and Pathogenesis of Amyotrophic Lateral Sclerosis: State of the Art and Research Perspectives

(2013) Francesca Trojsi et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Dexpramipexole versus placebo for patients with amyotrophic lateral sclerosis (EMPOWER): a randomised, double-blind, phase 3 trial

(2013) Merit E Cudkowicz et al.   LANCET NEUROLOGY

Polymerase chain reaction and Southern blot-based analysis of the C9orf72 hexanucleotide repeat in different motor neuron diseases

(2013) Annemarie Hübers et al.   NEUROBIOLOGY OF AGING

Adipose Tissue Distribution Predicts Survival in Amyotrophic Lateral Sclerosis

(2013) Eva Lindauer et al.   PLoS One

Structure and Ubiquitination-Dependent Activation of TANK-Binding Kinase 1

(2013) Daqi Tu et al.   Cell Reports

Crystal Structure and Mechanism of Activation of TANK-Binding Kinase 1

(2013) Amede Larabi et al.   Cell Reports

Body mass index (BMI) as predictor of ALSFRS-R score decline in ALS patients

(2013) Ronit Reich-Slotky et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Effect of Acute Extremely Low Frequency Electromagnetic Field Exposure on the Antioxidant Status and Lipid Levels in Rat Brain

(2012) Jesús Martínez-Sámano et al.   ARCHIVES OF MEDICAL RESEARCH

Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72

(2012) Johnathan Cooper-Knock et al.   BRAIN

Neural stem cells: therapeutic potential for neurodegenerative diseases

(2012) G. Gincberg et al.   BRITISH MEDICAL BULLETIN

Frontotemporal Lobar Degeneration

(2012) Rachel E. Seltman et al.   CNS DRUGS

Oropharyngeal dysphagia in amyotrophic lateral sclerosis alters quality of life

(2012) G. Paris et al.   JOURNAL OF ORAL REHABILITATION

Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study

(2012) Elisa Majounie et al.   LANCET NEUROLOGY

The C9orf72 hexanucleotide repeat expansion in FTD and ALS

(2012) Eileen H. Bigio   Nature Reviews Neurology

Regulation of FMO and PON Detoxication Systems in ALS Human Tissues

(2012) Stella Gagliardi et al.   NEUROTOXICITY RESEARCH

Association between Extremely Low-Frequency Electromagnetic Fields Occupations and Amyotrophic Lateral Sclerosis: A Meta-Analysis

(2012) Hongjie Zhou et al.   PLoS One

Antisense oligonucleotide therapeutics for inherited neurodegenerative diseases

(2012) Amber L. Southwell et al.   TRENDS IN MOLECULAR MEDICINE

The potential of GPNMB as novel neuroprotective factor in amyotrophic lateral sclerosis

(2012) Hirotaka Tanaka et al.   Scientific Reports

Premorbid body mass index and risk of amyotrophic lateral sclerosis

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

Polyubiquitin Binding to Optineurin Is Required for Optimal Activation of TANK-binding Kinase 1 and Production of Interferon β

(2011) Catherine E. Gleason et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TBK1 Directly Engages Akt/PKB Survival Signaling to Support Oncogenic Transformation

(2011) Yi-Hung Ou et al.   MOLECULAR CELL

Body mass index, not dyslipidemia, is an independent predictor of survival in amyotrophic lateral sclerosis

(2011) Sabrina Paganoni et al.   MUSCLE & NERVE

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

(2011) Laura Ferraiuolo et al.   Nature Reviews Neurology

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

Human Glial-Restricted Progenitor Transplantation into Cervical Spinal Cord of the SOD1G93A Mouse Model of ALS

(2011) Angelo C. Lepore et al.   PLoS One

Stem cell technology for the study and treatment of motor neuron diseases

(2011) J Simon Lunn et al.   Regenerative Medicine

TBK1 Mediates Crosstalk Between the Innate Immune Response and Autophagy

(2011) H. Weidberg et al.   Science Signaling

Current Challenges for the Advancement of Neural Stem Cell Biology and Transplantation Research

(2011) Kristien Reekmans et al.   Stem Cell Reviews and Reports

Association Between Blood Lead and the Risk of Amyotrophic Lateral Sclerosis

(2010) F. Fang et al.   AMERICAN JOURNAL OF EPIDEMIOLOGY

Paraoxonase gene mutations in amyotrophic lateral sclerosis

(2010) Nicola Ticozzi et al.   ANNALS OF NEUROLOGY

Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis

(2010) S. Byrne et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Chromosome 9p21 in amyotrophic lateral sclerosis in Finland: a genome-wide association study

(2010) Hannu Laaksovirta et al.   LANCET NEUROLOGY

Capturing VCP: Another Molecular Piece in the ALS Jigsaw Puzzle

(2010) Christopher E. Shaw   NEURON

Caloric Restriction Shortens Lifespan through an Increase in Lipid Peroxidation, Inflammation and Apoptosis in the G93A Mouse, an Animal Model of ALS

(2010) Barkha P. Patel et al.   PLoS One

Current and emerging treatments for amyotrophic lateral sclerosis

(2010) Zoccolella   Neuropsychiatric Disease and Treatment

Exposure to chemicals and metals and risk of amyotrophic lateral sclerosis: A systematic review

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

Microglia in ALS: The Good, The Bad, and The Resting

(2009) Jenny S. Henkel et al.   Journal of Neuroimmune Pharmacology

Incidence of amyotrophic lateral sclerosis in Europe

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

Intravenous Administration of Self-complementary AAV9 Enables Transgene Delivery to Adult Motor Neurons

(2009) Sandra Duque et al.   MOLECULAR THERAPY

The TBK1 adaptor and autophagy receptor NDP52 restricts the proliferation of ubiquitin-coated bacteria

(2009) Teresa L M Thurston et al.   NATURE IMMUNOLOGY

Amyotrophic lateral sclerosis

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

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model

(2008) Maged M. Harraz et al.   JOURNAL OF CLINICAL INVESTIGATION

Focal transplantation–based astrocyte replacement is neuroprotective in a model of motor neuron disease

(2008) Angelo C Lepore et al.   NATURE NEUROSCIENCE

Genetics of Amyotrophic Lateral Sclerosis

(2008) Nailah Siddique et al.   Physical Medicine and Rehabilitation Clinics of North America

T lymphocytes potentiate endogenous neuroprotective inflammation in a mouse model of ALS

(2008) I. M. Chiu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

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

Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease

(2007) Emanuele Buratti   Frontiers in Bioscience-Landmark