New Insights on the Mechanisms of Disease Course Variability in ALS from Mutant SOD1 Mouse Models

C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits

(2015) Max Koppers et al.   ANNALS OF NEUROLOGY

Prolyl isomerases in gene transcription

(2015) Steven D. Hanes   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Sporadic and hereditary amyotrophic lateral sclerosis (ALS)

(2015) Senda Ajroud-Driss et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Peptidylprolyl isomerase A governs TARDBP function and assembly in heterogeneous nuclear ribonucleoprotein complexes

(2015) Eliana Lauranzano et al.   BRAIN

Stratification of ALS patients’ survival: a population-based study

(2015) Benoît Marin et al.   JOURNAL OF NEUROLOGY

TBK1 mutation frequencies in French frontotemporal dementia and amyotrophic lateral sclerosis cohorts

(2015) Isabelle Le Ber et al.   NEUROBIOLOGY OF AGING

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

Endolysosomal Deficits Augment Mitochondria Pathology in Spinal Motor Neurons of Asymptomatic fALS Mice

(2015) Yuxiang Xie et al.   NEURON

Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice

(2015) Owen M. Peters et al.   NEURON

C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

(2015) Jacqueline G. O’Rourke et al.   NEURON

Amyotrophic lateral sclerosis: Current perspectives from basic research to the clinic

(2015) Renzo Mancuso et al.   PROGRESS IN NEUROBIOLOGY

Comparative Magnetic Resonance Imaging and Histopathological Correlates in Two SOD1 Transgenic Mouse Models of Amyotrophic Lateral Sclerosis

(2015) Ilaria Caron et al.   PLoS One

Quantifying disease progression in amyotrophic lateral sclerosis

(2014) Neil G. Simon et al.   ANNALS OF NEUROLOGY

Long-term survival in amyotrophic lateral sclerosis: A population-based study

(2014) Elisabetta Pupillo et al.   ANNALS OF NEUROLOGY

Fast axonal transport of the proteasome complex depends on membrane interaction and molecular motor function

(2014) M. G. Otero et al.   JOURNAL OF CELL SCIENCE

Clinical trials in amyotrophic lateral sclerosis: why so many negative trials and how can trials be improved?

(2014) Hiroshi Mitsumoto et al.   LANCET NEUROLOGY

Preclinical research: Make mouse studies work

(2014) Steve Perrin   NATURE

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

(2014) Janel O Johnson et al.   NATURE NEUROSCIENCE

ATF3 expression improves motor function in the ALS mouse model by promoting motor neuron survival and retaining muscle innervation

(2014) R. Seijffers et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Dietary ω-3 Polyunsaturated Fatty Acid Intake and Risk for Amyotrophic Lateral Sclerosis

(2014) Kathryn C. Fitzgerald et al.   JAMA Neurology

Rodent models of amyotrophic lateral sclerosis

(2013) Philip McGoldrick et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Transcriptomic indices of fast and slow disease progression in two mouse models of amyotrophic lateral sclerosis

(2013) Giovanni Nardo et al.   BRAIN

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

The Omega-3 Fatty Acid Eicosapentaenoic Acid Accelerates Disease Progression in a Model of Amyotrophic Lateral Sclerosis

(2013) Ping K. Yip et al.   PLoS One

C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice

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

Neurons as targets for T cells in the nervous system

(2013) Roland S. Liblau et al.   TRENDS IN NEUROSCIENCES

Effect of genetic background on onset and disease progression in the SOD1-G93A model of amyotrophic lateral sclerosis

(2012) Renzo Mancuso et al.   Amyotrophic Lateral Sclerosis

Mitochondria and ALS: Implications from novel genes and pathways

(2012) Mauro Cozzolino et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Plasma Neurofilament Heavy Chain Levels Correlate to Markers of Late Stage Disease Progression and Treatment Response in SOD1G93A Mice that Model ALS

(2012) Ching-Hua Lu et al.   PLoS One

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

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

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

Differential Involvement of Optineurin in Amyotrophic Lateral Sclerosis With or Without SOD1 Mutations

(2011) Han-Xiang Deng   ARCHIVES OF NEUROLOGY

SQSTM1 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

(2011) Faisal Fecto   ARCHIVES OF NEUROLOGY

Complement upregulation and activation on motor neurons and neuromuscular junction in the SOD1 G93A mouse model of familial amyotrophic lateral sclerosis

(2011) Bianca Heurich et al.   JOURNAL OF NEUROIMMUNOLOGY

Wallerian degeneration: gaining perspective on inflammatory events after peripheral nerve injury

(2011) Andrew D Gaudet et al.   Journal of Neuroinflammation

SOD1 and TDP-43 animal models of amyotrophic lateral sclerosis: recent advances in understanding disease toward the development of clinical treatments

(2011) Peter I. Joyce et al.   MAMMALIAN GENOME

SOD1 G93D sporadic amyotrophic lateral sclerosis (SALS) patient with rapid progression and concomitant novel ANG variant

(2011) Marco Luigetti et al.   NEUROBIOLOGY OF AGING

In Vivo Imaging Reveals Distinct Inflammatory Activity of CNS Microglia versus PNS Macrophages in a Mouse Model for ALS

(2011) Payam Dibaj et al.   PLoS One

Amyotrophic Lateral Sclerosis Multiprotein Biomarkers in Peripheral Blood Mononuclear Cells

(2011) Giovanni Nardo et al.   PLoS One

ALS genetic modifiers that increase survival of SOD1 mice and are suitable for therapeutic development

(2011) Giulietta Riboldi et al.   PROGRESS IN NEUROBIOLOGY

Dysfunction of constitutive and inducible ubiquitin-proteasome system in amyotrophic lateral sclerosis: Implication for protein aggregation and immune response

(2011) Caterina Bendotti et al.   PROGRESS IN NEUROBIOLOGY

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

Guidelines for preclinical animal research in ALS/MND: A consensus meeting

(2010) Albert C. Ludolph et al.   Amyotrophic Lateral Sclerosis

A Systems Biology Approach Identifies Inflammatory Abnormalities Between Mouse Strains Prior to Development of Metabolic Disease

(2010) M. A. Mori et al.   DIABETES

Chloride intracellular channel 1 (CLIC1): Sensor and effector during oxidative stress

(2010) Stefania Averaimo et al.   FEBS LETTERS

The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS)

(2010) Valeria Crippa et al.   HUMAN MOLECULAR GENETICS

Transgenic mice with enhanced neuronal major histocompatibility complex class I expression recover locomotor function better after spinal cord injury

(2010) M. Selvan Joseph et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Energy metabolism in amyotrophic lateral sclerosis

(2010) Luc Dupuis et al.   LANCET NEUROLOGY

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

Lithium carbonate in amyotrophic lateral sclerosis: Lack of efficacy in a dose-finding trial

(2010) A. Chio et al.   NEUROLOGY

Mutations in CHMP2B in Lower Motor Neuron Predominant Amyotrophic Lateral Sclerosis (ALS)

(2010) Laura E. Cox et al.   PLoS One

Genetic polymorphisms among C57BL/6 mouse inbred strains

(2010) Esther Zurita et al.   TRANSGENIC RESEARCH

Complement in neuroprotection and neurodegeneration

(2010) Vijay Yanamadala et al.   TRENDS IN MOLECULAR MEDICINE

Treatment with lithium carbonate does not improve disease progression in two different strains of SOD1 mutant mice

(2009) Chiara Pizzasegola et al.   Amyotrophic Lateral Sclerosis

Prognostic factors in ALS: A critical review

(2009) Adriano Chiò et al.   Amyotrophic Lateral Sclerosis

Mitochondrial Function, Morphology, and Axonal Transport in Amyotrophic Lateral Sclerosis

(2009) Jordi Magrané et al.   ANTIOXIDANTS & REDOX SIGNALING

Nitroproteomics of Peripheral Blood Mononuclear Cells from Patients and a Rat Model of ALS

(2009) Giovanni Nardo et al.   ANTIOXIDANTS & REDOX SIGNALING

Neuromuscular junction destruction during amyotrophic lateral sclerosis: insights from transgenic models

(2009) Luc Dupuis et al.   CURRENT OPINION IN PHARMACOLOGY

Immunoreactivity of the phosphorylated axonal neurofilament H subunit (pNF-H) in blood of ALS model rodents and ALS patients: evaluation of blood pNF-H as a potential ALS biomarker

(2009) Kevin Boylan et al.   JOURNAL OF NEUROCHEMISTRY

Classical Major Histocompatibility Complex Class I Molecules in Motoneurons: New Actors at the Neuromuscular Junction

(2009) S. Thams et al.   JOURNAL OF NEUROSCIENCE

No Benefit from Chronic Lithium Dosing in a Sibling-Matched, Gender Balanced, Investigator-Blinded Trial Using a Standard Mouse Model of Familial ALS

(2009) Alan Gill et al.   PLoS One

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

Activation of innate and humoral immunity in the peripheral nervous system of ALS transgenic mice

(2009) I. M. Chiu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Design, power, and interpretation of studies in the standard murine model of ALS

(2008) Sean Scott et al.   Amyotrophic Lateral Sclerosis

A novel mutation in thesenataxingene identified in a Chinese patient with sporadic amyotrophic lateral sclerosis

(2008) Zhen-hua Zhao et al.   Amyotrophic Lateral Sclerosis

Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity

(2008) Gabriella Dobrowolny et al.   Cell Metabolism

An Animal Model of Oral Dysphagia in Amyotrophic Lateral Sclerosis

(2008) Teresa E. Lever et al.   DYSPHAGIA

Requirement of Myeloid Cells for Axon Regeneration

(2008) B. Barrette et al.   JOURNAL OF NEUROSCIENCE

CLIC1 Function Is Required for  -Amyloid-Induced Generation of Reactive Oxygen Species by Microglia

(2008) R. H. Milton et al.   JOURNAL OF NEUROSCIENCE

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS

SOD1 and Amyotrophic Lateral Sclerosis: Mutations and Oligomerization

(2008) Lucia Banci et al.   PLoS One

Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS

(2008) B TURNER et al.   PROGRESS IN NEUROBIOLOGY