Amyotrophic lateral sclerosis

The changing picture of amyotrophic lateral sclerosis: lessons from European registers

(2017) Orla Hardiman et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Edaravone: a new treatment for ALS on the horizon?

(2017) Orla Hardiman et al.   LANCET NEUROLOGY

Pain in amyotrophic lateral sclerosis

(2017) Adriano Chiò et al.   LANCET NEUROLOGY

Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial

(2017) Koji Abe et al.   LANCET NEUROLOGY

Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics

(2017) Steven Boeynaems et al.   MOLECULAR CELL

C9orf72 expansion disrupts ATM-mediated chromosomal break repair

(2017) Callum Walker et al.   NATURE NEUROSCIENCE

Benefits, pitfalls, and future design of population-based registers in neurodegenerative disease

(2017) James P.K. Rooney et al.   NEUROLOGY

Genetic testing in ALS

(2017) Alice Vajda et al.   NEUROLOGY

Enhanced Bulbar Function in Amyotrophic Lateral Sclerosis: The Nuedexta Treatment Trial

(2017) Richard Smith et al.   Neurotherapeutics

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

SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits

(2017) Guillaume M. Hautbergue et al.   Nature Communications

Comparison of the King’s and MiToS staging systems for ALS

(2017) Ton Fang et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Prognostic value of clinical and electrodiagnostic parameters at time of diagnosis in patients with amyotrophic lateral sclerosis

(2017) Wendeline Reniers et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria

(2017) Michael J. Strong et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Diagnostic and Prognostic Biomarkers in Amyotrophic Lateral Sclerosis

(2017) Alessandra Gaiani et al.   JAMA Neurology

A Cross-sectional population-based investigation into behavioral change in amyotrophic lateral sclerosis: subphenotypes, staging, cognitive predictors, and survival

(2017) Tom Burke et al.   Annals of Clinical and Translational Neurology

Botulinum toxin-A injections vs radiotherapy for drooling in ALS

(2016) J. G. Weikamp et al.   ACTA NEUROLOGICA SCANDINAVICA

Defective recognition of LC3B by mutant SQSTM1/p62 implicates impairment of autophagy as a pathogenic mechanism in ALS-FTLD

(2016) Alice Goode et al.   Autophagy

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome

(2016) Roland Hjerpe et al.   CELL

TDP‐43 loss of function inhibits endosomal trafficking and alters trophic signaling in neurons

(2016) Benjamin M Schwenk et al.   EMBO JOURNAL

The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy

(2016) Christopher P Webster et al.   EMBO JOURNAL

ALS/FTD‐associated FUS activates GSK‐3β to disrupt the VAPB–PTPIP51 interaction and ER–mitochondria associations

(2016) Radu Stoica et al.   EMBO REPORTS

Amyotrophic lateral sclerosis: a comparison of two staging systems in a population-based study

(2016) D. Ferraro et al.   EUROPEAN JOURNAL OF NEUROLOGY

The Use of Peripherally Inserted Central Catheter in Amyotrophic Lateral Sclerosis Patients at a Later Stage

(2016) Raul Juntas-Morales et al.   EUROPEAN NEUROLOGY

Genetic testing and genetic counseling for amyotrophic lateral sclerosis: an update for clinicians

(2016) Jennifer Roggenbuck et al.   GENETICS IN MEDICINE

Functional links between SQSTM1 and ALS2 in the pathogenesis of ALS: cumulative impact on the protection against mutant SOD1-mediated motor dysfunction in mice

(2016) Shinji Hadano et al.   HUMAN MOLECULAR GENETICS

Performance predictors of brain–computer interfaces in patients with amyotrophic lateral sclerosis

(2016) A Geronimo et al.   Journal of Neural Engineering

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

(2016) Antonia Ratti et al.   JOURNAL OF NEUROCHEMISTRY

Amyotrophic lateral sclerosis: moving towards a new classification system

(2016) Ammar Al-Chalabi et al.   LANCET NEUROLOGY

NEK1 variants confer susceptibility to amyotrophic lateral sclerosis

(2016) Kevin P Kenna et al.   NATURE GENETICS

Genome-wide association analyses identify new risk variants and the genetic architecture of amyotrophic lateral sclerosis

(2016) Wouter van Rheenen et al.   NATURE GENETICS

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

(2016) Wenzhang Wang et al.   NATURE MEDICINE

Active zone scaffolds differentially accumulate Unc13 isoforms to tune Ca2+ channel–vesicle coupling

(2016) Mathias A Böhme et al.   NATURE NEUROSCIENCE

Gene discovery in amyotrophic lateral sclerosis: implications for clinical management

(2016) Ammar Al-Chalabi et al.   Nature Reviews Neurology

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

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

Presymptomatic ALS genetic counseling and testing

(2016) Michael Benatar et al.   NEUROLOGY

A randomized trial of mexiletine in ALS

(2016) Michael D. Weiss et al.   NEUROLOGY

Death with dignity in Washington patients with amyotrophic lateral sclerosis

(2016) Leo H. Wang et al.   NEUROLOGY

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

Metabolic Biomarkers and Neurodegeneration: A Pathway Enrichment Analysis of Alzheimer's Disease, Parkinson's Disease, and Amyotrophic Lateral Sclerosis

(2016) Medi Kori et al.   OMICS-A JOURNAL OF INTEGRATIVE BIOLOGY

Oligodendrocytes contribute to motor neuron death in ALS via SOD1-dependent mechanism

(2016) Laura Ferraiuolo et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ALS Mutations Disrupt Phase Separation Mediated by α-Helical Structure in the TDP-43 Low-Complexity C-Terminal Domain

(2016) Alexander E. Conicella et al.   STRUCTURE

Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses

(2016) Katannya Kapeli et al.   Nature Communications

Projected increase in amyotrophic lateral sclerosis from 2015 to 2040

(2016) Karissa C. Arthur et al.   Nature Communications

Riluzole 5 mg/mL oral suspension: for optimized drug delivery in amyotrophic lateral sclerosis

(2016) Ann Margaret Dyer et al.   Drug Design Development and Therapy

Screening for cognitive dysfunction in ALS: validation of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) using age and education adjusted normative data

(2016) Marta Pinto-Grau et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Rare genetic variation in UNC13A may modify survival in amyotrophic lateral sclerosis

(2016) Benjamin Gaastra et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

National Study of Muscle Cramps in ALS in the USA

(2016) Helen E. Stephens et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Correlation between Forced Vital Capacity and Slow Vital Capacity for the assessment of respiratory involvement in Amyotrophic Lateral Sclerosis: a prospective study

(2016) Susana Pinto et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

The selective anatomical vulnerability of ALS: ‘disease-defining’ and ‘disease-defying’ brain regions

(2016) Peter Bede et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

The NEK1 interactor, C21ORF2, is required for efficient DNA damage repair

(2015) Xiao Fang et al.   ACTA BIOCHIMICA ET BIOPHYSICA SINICA

Brain–computer interfaces in amyotrophic lateral sclerosis: A metanalysis

(2015) Mauro Marchetti et al.   CLINICAL NEUROPHYSIOLOGY

CHCHD10 mutations promote loss of mitochondrial cristae junctions with impaired mitochondrial genome maintenance and inhibition of apoptosis

(2015) E. C. Genin et al.   EMBO Molecular Medicine

Clinical and demographic factors and outcome of amyotrophic lateral sclerosis in relation to population ancestral origin

(2015) Benoît Marin et al.   EUROPEAN JOURNAL OF EPIDEMIOLOGY

p62/SQSTM1 functions as a signaling hub and an autophagy adaptor

(2015) Yoshinori Katsuragi et al.   FEBS Journal

TBK1 controls autophagosomal engulfment of polyubiquitinated mitochondria through p62/SQSTM1 phosphorylation

(2015) Gen Matsumoto et al.   HUMAN MOLECULAR GENETICS

Prognostic Role of Serum Levels of Uric Acid in Amyotrophic Lateral Sclerosis

(2015) Seong-il Oh et al.   Journal of Clinical Neurology

Inclusion body myositis: A review of clinical and genetic aspects, diagnostic criteria and therapeutic approaches

(2015) Frank L. Mastaglia et al.   JOURNAL OF CLINICAL NEUROSCIENCE

Predicting prognosis in amyotrophic lateral sclerosis: a simple algorithm

(2015) Marwa Elamin et al.   JOURNAL OF NEUROLOGY

Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: a prospective observational study

(2015) Johannes Dorst et al.   JOURNAL OF NEUROLOGY

Caregiver burden in amyotrophic lateral sclerosis: a cross-sectional investigation of predictors

(2015) Tom Burke et al.   JOURNAL OF NEUROLOGY

Presymptomatic and longitudinal neuroimaging in neurodegeneration—from snapshots to motion picture: a systematic review

(2015) Christina Schuster et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Gastrostomy in patients with amyotrophic lateral sclerosis (ProGas): a prospective cohort study

(2015)   LANCET NEUROLOGY

Quality of life in amyotrophic lateral sclerosis patients and caregivers: Impact of assistive communication from early stages

(2015) Ana Londral et al.   MUSCLE & NERVE

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport

(2015) Ke Zhang et al.   NATURE

GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

(2015) Brian D. Freibaum et al.   NATURE

R loops: new modulators of genome dynamics and function

(2015) José M. Santos-Pereira et al.   NATURE REVIEWS GENETICS

Differences in protein quality control correlate with phenotype variability in 2 mouse models of familial amyotrophic lateral sclerosis

(2015) Marianna Marino et al.   NEUROBIOLOGY OF AGING

18F-FDG-PET correlates of cognitive impairment in ALS

(2015) Antonio Canosa et al.   NEUROLOGY

Invited Review: Decoding the pathophysiological mechanisms that underlie RNA dysregulation in neurodegenerative disorders: a review of the current state of the art

(2015) Matthew J. Walsh et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Defective Proteasome Delivery of Polyubiquitinated Proteins by Ubiquilin-2 Proteins Containing ALS Mutations

(2015) Lydia Chang et al.   PLoS One

A preliminary randomized trial of the mechanical insufflator-exsufflator versus breath-stacking technique in patients with amyotrophic lateral sclerosis

(2015) Muhammad K. Rafiq et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Receiving the news of a diagnosis of motor neuron disease: What does it take to make it better?

(2015) Samar M Aoun et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

A revision of the El Escorial criteria - 2015

(2015) Albert Ludolph et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Prion-like Mechanism in Amyotrophic Lateral Sclerosis: are Protein Aggregates the Key?

(2015) Shynrye Lee et al.   Experimental Neurobiology

Genetic Background Effects on Disease Onset and Lifespan of the Mutant Dynactin p150Glued Mouse Model of Motor Neuron Disease

(2015) Terry D. Heiman-Patterson et al.   PLoS One

C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral Sclerosis

(2015) Johnathan Cooper-Knock et al.   PLoS One

Functional Connectivity Changes in Resting-State EEG as Potential Biomarker for Amyotrophic Lateral Sclerosis

(2015) Parameswaran Mahadeva Iyer et al.   PLoS One

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

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

Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions

(2014) Johnathan Cooper-Knock et al.   BRAIN

A mitochondrial origin for frontotemporal dementia and amyotrophic lateral sclerosis through CHCHD10 involvement

(2014) Sylvie Bannwarth et al.   BRAIN

Cell to Cell Spreading of Misfolded Proteins as a Therapeutic Target in Motor Neuron Disease

(2014) Livia Pasquali et al.   CURRENT MEDICINAL CHEMISTRY

Population-based epidemiology of amyotrophic lateral sclerosis (ALS) in an ageing Europe - the French register of ALS in Limousin (FRALim register)

(2014) B. Marin et al.   EUROPEAN JOURNAL OF NEUROLOGY

Response shift recalibration and reprioritization in health-related quality of life was identified prospectively in older men with and without stroke

(2014) Ruth Barclay et al.   JOURNAL OF CLINICAL EPIDEMIOLOGY

A multidisciplinary clinic approach improves survival in ALS: a comparative study of ALS in Ireland and Northern Ireland

(2014) James Rooney et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

C9orf72 nucleotide repeat structures initiate molecular cascades of disease

(2014) Aaron R. Haeusler et al.   NATURE

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

(2014) Janel O Johnson et al.   NATURE NEUROSCIENCE

Venous thromboembolism in amyotrophic lateral sclerosis: A prospective study

(2014) M. Gladman et al.   NEUROLOGY

Exome-wide Rare Variant Analysis Identifies TUBA4A Mutations Associated with Familial ALS

(2014) Bradley N. Smith et al.   NEURON

Patient-Perceived Outcomes and Quality of Life in ALS

(2014) Zachary Simmons   Neurotherapeutics

Optineurin is an autophagy receptor for damaged mitochondria in parkin-mediated mitophagy that is disrupted by an ALS-linked mutation

(2014) Yvette C. Wong et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mechanisms of genome instability induced by RNA-processing defects

(2014) Yujia A. Chan et al.   TRENDS IN GENETICS

Microglia centered pathogenesis in ALS: insights in cell interconnectivity

(2014) Dora Brites et al.   Frontiers in Cellular Neuroscience

Superoxide dismutase 1 acts as a nuclear transcription factor to regulate oxidative stress resistance

(2014) Chi Kwan Tsang et al.   Nature Communications

RandomForest4Life: A Random Forest for predicting ALS disease progression

(2014) Torsten Hothorn et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

The El Escorial criteria: Strengths and weaknesses

(2014) Federica Agosta et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Estimating clinical stage of amyotrophic lateral sclerosis from the ALS Functional Rating Scale

(2014) Rubika Balendra et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Amyotrophic Lateral Sclerosis Outcome Measures and the Role of Albumin and Creatinine

(2014) Adriano Chiò et al.   JAMA Neurology

Lessons of ALS imaging: Pitfalls and future directions — A critical review

(2014) Peter Bede et al.   NeuroImage-Clinical

ERBB4 Mutations that Disrupt the Neuregulin-ErbB4 Pathway Cause Amyotrophic Lateral Sclerosis Type 19

(2013) Yuji Takahashi et al.   AMERICAN JOURNAL OF HUMAN GENETICS

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

Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis

(2013) Thomas Philips et al.   BRAIN

Role of Stress Granules and RNA-Binding Proteins in Neurodegeneration: A Mini-Review

(2013) Tara Vanderweyde et al.   GERONTOLOGY

Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models

(2013) Jordi Magrané et al.   HUMAN MOLECULAR GENETICS

The Disease-associated r(GGGGCC)nRepeat from theC9orf72Gene Forms Tract Length-dependent Uni- and Multimolecular RNA G-quadruplex Structures

(2013) Kaalak Reddy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ribonuclease/angiogenin inhibitor 1 regulates stress-induced subcellular localization of angiogenin to control growth and survival

(2013) E. Pizzo et al.   JOURNAL OF CELL SCIENCE

Development and evaluation of a clinical staging system for amyotrophic lateral sclerosis

(2013) Adriano Chiò et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Genetic counselling in ALS: facts, uncertainties and clinical suggestions

(2013) A. Chio et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Enhancing Mitochondrial Calcium Buffering Capacity Reduces Aggregation of Misfolded SOD1 and Motor Neuron Cell Death without Extending Survival in Mouse Models of Inherited Amyotrophic Lateral Sclerosis

(2013) P. A. Parone et al.   JOURNAL OF NEUROSCIENCE

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis

(2013) Shin H Kang et al.   NATURE NEUROSCIENCE

Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons

(2013) Wen-Yuan Wang et al.   NATURE NEUROSCIENCE

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

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

(2013) A. Chiò et al.   NEUROEPIDEMIOLOGY

Cognitive changes predict functional decline in ALS: A population-based longitudinal study

(2013) M. Elamin et al.   NEUROLOGY

RNA Toxicity from the ALS/FTD C9ORF72 Expansion Is Mitigated by Antisense Intervention

(2013) Christopher J. Donnelly et al.   NEURON

Molecular Imaging of Microglial Activation in Amyotrophic Lateral Sclerosis

(2013) Philippe Corcia et al.   PLoS One

Increased Neurofilament Light Chain Blood Levels in Neurodegenerative Neurological Diseases

(2013) Johanna Gaiottino et al.   PLoS One

ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43

(2013) E. S. Arnold et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Hexanucleotide Repeats in ALS/FTD Form Length-Dependent RNA Foci, Sequester RNA Binding Proteins, and Are Neurotoxic

(2013) Youn-Bok Lee et al.   Cell Reports

Is exposure to cyanobacteria an environmental risk factor for amyotrophic lateral sclerosis and other neurodegenerative diseases?

(2013) Walter G. Bradley et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Screening for cognition and behaviour changes in ALS

(2013) Sharon Abrahams et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Incidence of Amyotrophic Lateral Sclerosis Among American Indians and Alaska Natives

(2013) Paul H. Gordon et al.   JAMA Neurology

Amyotrophic lateral sclerosis: Time for research on psychological intervention?

(2012) Francesco Pagnini et al.   Amyotrophic Lateral Sclerosis

Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS

(2012) Bing Liao et al.   EXPERIMENTAL NEUROLOGY

Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis

(2012) Julien Couthouis et al.   HUMAN MOLECULAR GENETICS

Evidence for an oligogenic basis of amyotrophic lateral sclerosis

(2012) Marka van Blitterswijk et al.   HUMAN MOLECULAR GENETICS

Oligodendroglia metabolically support axons and contribute to neurodegeneration

(2012) Youngjin Lee et al.   NATURE

Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis

(2012) Chi-Hong Wu et al.   NATURE

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

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

Speech therapy and communication device: Impact on quality of life and mood in patients with amyotrophic lateral sclerosis

(2012) Sonja Körner et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Incidence of amyotrophic lateral sclerosis in the Faroe Islands

(2011) P. Joensen   ACTA NEUROLOGICA SCANDINAVICA

Effect of genetic background on phenotype variability in transgenic mouse models of amyotrophic lateral sclerosis: A window of opportunity in the search for genetic modifiers

(2011) Terry D. Heiman-Patterson et al.   Amyotrophic Lateral Sclerosis

Proposed criteria for familial amyotrophic lateral sclerosis

(2011) Susan Byrne et al.   Amyotrophic Lateral Sclerosis

A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis

(2011) Amr Al-Saif et al.   ANNALS OF NEUROLOGY

SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2011) Faisal Fecto   ARCHIVES OF NEUROLOGY

The Seeds of Neurodegeneration: Prion-like Spreading in ALS

(2011) Magdalini Polymenidou et al.   CELL

EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) - revised report of an EFNS task force

(2011) et al.   EUROPEAN JOURNAL OF NEUROLOGY

VAPB interacts with the mitochondrial protein PTPIP51 to regulate calcium homeostasis

(2011) Kurt J. De Vos et al.   HUMAN MOLECULAR GENETICS

Onset and spreading patterns of lower motor neuron involvements predict survival in sporadic amyotrophic lateral sclerosis

(2011) C. Fujimura-Kiyono et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Non-invasive ventilation in amyotrophic lateral sclerosis: a 10 year population based study

(2011) Adriano Chiò et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study

(2011) Julie Phukan et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Regulation of Oligodendrocyte Development and Myelination by Glucose and Lactate

(2011) J. E. Rinholm et al.   JOURNAL OF NEUROSCIENCE

Amyotrophic lateral sclerosis

(2011) Matthew C Kiernan et al.   LANCET

Human Senataxin Resolves RNA/DNA Hybrids Formed at Transcriptional Pause Sites to Promote Xrn2-Dependent Termination

(2011) Konstantina Skourti-Stathaki et al.   MOLECULAR CELL

Botulinum toxin A versus B in sialorrhea: A prospective, randomized, double-blind, crossover pilot study in patients with amyotrophic lateral sclerosis or Parkinson's disease

(2011) Arianna Guidubaldi et al.   MOVEMENT DISORDERS

Coping strategies in relation to quality of life in amyotrophic lateral sclerosis

(2011) Sébastien Montel 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

Selective Neuronal Vulnerability in Neurodegenerative Diseases: from Stressor Thresholds to Degeneration

(2011) Smita Saxena et al.   NEURON

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

Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset

(2011) Christine Vande Velde et al.   PLoS One

TBK1 Mediates Crosstalk Between the Innate Immune Response and Autophagy

(2011) H. Weidberg et al.   Science Signaling

Cognitive and behavioral challenges in caring for patients with frontotemporal dementia and amyotrophic lateral sclerosis

(2010) Jennifer Merrilees et al.   Amyotrophic Lateral Sclerosis

Psychological morbidity in ALS: The importance of psychological assessment beyond depression alone

(2010) Stephanie H. Felgoise et al.   Amyotrophic Lateral Sclerosis

Safety of home parenteral nutrition in patients with amyotrophic lateral sclerosis: A French national survey

(2010) Maya Abdelnour-Mallet et al.   Amyotrophic Lateral Sclerosis

The epidemiology and treatment of ALS: Focus on the heterogeneity of the disease and critical appraisal of therapeutic trials

(2010) Ettore Beghi et al.   Amyotrophic Lateral Sclerosis

Dextromethorphan Plus Ultra Low-Dose Quinidine Reduces Pseudobulbar Affect

(2010) Erik P. Pioro et al.   ANNALS OF NEUROLOGY

Sigma nonopioid intracellular receptor 1 mutations cause frontotemporal lobar degeneration-motor neuron disease

(2010) Agnes A. Luty et al.   ANNALS OF NEUROLOGY

Structure and function of mRNA export adaptors

(2010) Matthew J. Walsh et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice

(2010) L. Wang et al.   HUMAN MOLECULAR GENETICS

Characterization of the Properties of a Novel Mutation in VAPB in Familial Amyotrophic Lateral Sclerosis

(2010) Han-Jou Chen et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Superoxide dismutase-1 and other proteins in inclusions from transgenic amyotrophic lateral sclerosis model mice

(2010) Daniel Bergemalm et al.   JOURNAL OF NEUROCHEMISTRY

The diagnostic pathway and prognosis in bulbar-onset amyotrophic lateral sclerosis

(2010) Martin R. Turner et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Energy metabolism in amyotrophic lateral sclerosis

(2010) Luc Dupuis et al.   LANCET NEUROLOGY

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

(2010) Andrew C. Elden et al.   NATURE

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

TAR DNA-binding protein 43 in neurodegenerative disease

(2010) Alice S. Chen-Plotkin et al.   Nature Reviews Neurology

A novel peripherin gene (PRPH) mutation identified in one sporadic amyotrophic lateral sclerosis patient

(2010) Lucia Corrado et al.   NEUROBIOLOGY OF AGING

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

Loss of ALS2/Alsin Exacerbates Motor Dysfunction in a SOD1H46R-Expressing Mouse ALS Model by Disturbing Endolysosomal Trafficking

(2010) Shinji Hadano et al.   PLoS One

Deficits in axonal transport precede ALS symptoms in vivo

(2010) L. G. Bilsland et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Deleterious Variants of FIG4, a Phosphoinositide Phosphatase, in Patients with ALS

(2009) Clement Y. Chow et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Defective autophagy in neurons and astrocytes from mice deficient in PI(3,5)P2

(2009) Cole J. Ferguson et al.   HUMAN MOLECULAR GENETICS

Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease

(2009) Jeong-Sun Ju et al.   JOURNAL OF CELL BIOLOGY

Incidence of amyotrophic lateral sclerosis in Europe

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

Randomized double-blind study of botulinum toxin type B for sialorrhea in als patients

(2009) Carlayne E. Jackson et al.   MUSCLE & NERVE

Trends and determinants of end-of-life practices in ALS in the Netherlands

(2009) M. Maessen et al.   NEUROLOGY

Reduced frequency of ALS in an ethnically mixed population: A population-based mortality study

(2009) T. Zaldivar et al.   NEUROLOGY

Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Drug, nutritional, and respiratory therapies (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology

(2009) R. G. Miller et al.   NEUROLOGY

The Transcellular Spread of Cytosolic Amyloids, Prions, and Prionoids

(2009) Adriano Aguzzi et al.   NEURON

Characterization of Detergent-Insoluble Proteins in ALS Indicates a Causal Link between Nitrative Stress and Aggregation in Pathogenesis

(2009) Manuela Basso et al.   PLoS One

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

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

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

Gcn5- and Elp3-induced histone H3 acetylation regulateshsp70gene transcription in yeast

(2008) Qiuju Han et al.   BIOCHEMICAL JOURNAL

Variants of the elongator protein 3 ( ELP3 ) gene are associated with motor neuron degeneration

(2008) Claire L. Simpson et al.   HUMAN MOLECULAR GENETICS

Disturbance of Nuclear and Cytoplasmic TAR DNA-binding Protein (TDP-43) Induces Disease-like Redistribution, Sequestration, and Aggregate Formation

(2008) Matthew J. Winton et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Motor Neuron Disease Occurring in a Mutant Dynactin Mouse Model Is Characterized by Defects in Vesicular Trafficking

(2008) F. M. Laird et al.   JOURNAL OF NEUROSCIENCE

Preferential motor unit loss in the SOD1G93Atransgenic mouse model of amyotrophic lateral sclerosis

(2008) J. Hegedus et al.   JOURNAL OF PHYSIOLOGY-LONDON

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

The ALS/PDC syndrome of Guam and the cycad hypothesis

(2008) J. C. Steele et al.   NEUROLOGY

TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

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