Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP‐43 pathology

The Timing and Extent of Motor Neuron Vulnerability in ALS Correlates with Accumulation of Misfolded SOD1 Protein in the Cortex and in the Spinal Cord

(2020) Baris Genc et al.   Cells

The Electrophysiological Determinants of Corticospinal Motor Neuron Vulnerability in ALS

(2020) Javier H. Jara et al.   Frontiers in Molecular Neuroscience

Cortical Circuit Dysfunction as a Potential Driver of Amyotrophic Lateral Sclerosis

(2020) Aurore Brunet et al.   Frontiers in Neuroscience

Amyotrophic Lateral Sclerosis: Fuel for the Corticofugal Feud

(2020) Brian J. Wainger et al.   ANNALS OF NEUROLOGY

Absence of Subcerebral Projection Neurons Is Beneficial in a Mouse Model of Amyotrophic Lateral Sclerosis

(2020) Thibaut Burg et al.   ANNALS OF NEUROLOGY

The human motor cortex microcircuit: insights for neurodegenerative disease

(2020) Peter McColgan et al.   NATURE REVIEWS NEUROSCIENCE

Exciting Complexity: The Role of Motor Circuit Elements in ALS Pathophysiology

(2020) Zeynep I. Gunes et al.   Frontiers in Neuroscience

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

(2020) Chien-Hsiung Yu et al.   CELL

Expanded access: opening doors to personalized medicine for rare disease patients and patients with neurodegenerative diseases

(2020) P.Hande Ozdinler   FEBS Journal

Improving clinical trial outcomes in amyotrophic lateral sclerosis

(2020) Matthew C. Kiernan et al.   Nature Reviews Neurology

Evidence that corticofugal propagation of ALS pathology is not mediated by prion-like mechanism

(2020) Jelena Scekic-Zahirovic et al.   PROGRESS IN NEUROBIOLOGY

Transgenic and physiological mouse models give insights into different aspects of amyotrophic lateral sclerosis

(2019) Francesca De Giorgio et al.   Disease Models & Mechanisms

The molecular tweezer CLR01 inhibits aberrant superoxide dismutase 1 (SOD1) self-assembly in vitro and in the G93A-SOD1 mouse model of ALS

(2019) Ravinder Malik et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Misfolded SOD1 inclusions in patients with mutations in C9orf72 and other ALS/FTD-associated genes

(2019) Karin Forsberg et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Disruption of RNA Metabolism in Neurological Diseases and Emerging Therapeutic Interventions

(2019) Julia K. Nussbacher et al.   NEURON

Mouse models of neurodegeneration: Know your question, know your mouse

(2019) Elizabeth M. C. Fisher et al.   Science Translational Medicine

The Novel Small Molecule TRVA242 Stabilizes Neuromuscular Junction Defects in Multiple Animal Models of Amyotrophic Lateral Sclerosis

(2019) Poulomee Bose et al.   Neurotherapeutics

Complexity of Generating Mouse Models to Study the Upper Motor Neurons: Let Us Shift Focus from Mice to Neurons

(2019) Baris Genc et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Motor Neuron Susceptibility in ALS/FTD

(2019) Audrey M. G. Ragagnin et al.   Frontiers in Neuroscience

Edaravone, a free radical scavenger, protects against ferroptotic cell death in vitro

(2019) Takujiro Homma et al.   EXPERIMENTAL CELL RESEARCH

MCP1-CCR2 and neuroinflammation in the ALS motor cortex with TDP-43 pathology

(2019) Javier H. Jara et al.   Journal of Neuroinflammation

Modeling cell-autonomous motor neuron phenotypes in ALS using iPSCs

(2019) James Hawrot et al.   NEUROBIOLOGY OF DISEASE

Mitoautophagy: A Unique Self-Destructive Path Mitochondria of Upper Motor Neurons With TDP-43 Pathology Take, Very Early in ALS

(2019) Mukesh Gautam et al.   Frontiers in Cellular Neuroscience

Prion-Like Propagation of Protein Misfolding and Aggregation in Amyotrophic Lateral Sclerosis

(2019) Luke McAlary et al.   Frontiers in Molecular Neuroscience

SMN complex member Gemin3 self-interacts and has a functional relationship with ALS-linked proteins TDP-43, FUS and Sod1

(2019) Rebecca Cacciottolo et al.   Scientific Reports

Mouse models of ALS: Past, present and future

(2018) Cathleen Lutz   BRAIN RESEARCH

Incorporating upper motor neuron health in ALS drug discovery

(2018) Ina Dervishi et al.   DRUG DISCOVERY TODAY

TDP-43 interacts with mitochondrial proteins critical for mitophagy and mitochondrial dynamics

(2018) Stephani A. Davis et al.   NEUROSCIENCE LETTERS

ALS-related human cortical and motor neurons survival is differentially affected by Sema3A

(2018) Anastasya Birger et al.   Cell Death & Disease

Endoplasmic reticulum and mitochondria in diseases of motor and sensory neurons: a broken relationship?

(2018) Nathalie Bernard-Marissal et al.   Cell Death & Disease

Animal models of neurodegenerative diseases

(2018) Ted M. Dawson et al.   NATURE NEUROSCIENCE

Endoplasmic reticulum stress leads to accumulation of wild-type SOD1 aggregates associated with sporadic amyotrophic lateral sclerosis

(2018) Danilo B. Medinas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondria, ER, and nuclear membrane defects reveal early mechanisms for upper motor neuron vulnerability with respect to TDP-43 pathology

(2018) Mukesh Gautam et al.   ACTA NEUROPATHOLOGICA

All (animal) models (of neurodegeneration) are wrong. Are they also useful?

(2018) Richard M. Ransohoff   JOURNAL OF EXPERIMENTAL MEDICINE

Motor neuron degeneration, severe myopathy and TDP-43 increase in a transgenic pig model of SOD1-linked familiar ALS

(2018) Paola Crociara et al.   NEUROBIOLOGY OF DISEASE

Evidence for an early innate immune response in the motor cortex of ALS

(2017) Javier H. Jara et al.   Journal of Neuroinflammation

Clinical Significance of TDP-43 Neuropathology in Amyotrophic Lateral Sclerosis

(2017) Matthew D. Cykowski et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL 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

ER stress and the unfolded protein response in neurodegeneration

(2017) Claudio Hetz et al.   Nature Reviews Neurology

Mouse models of frontotemporal dementia: A comparison of phenotypes with clinical symptomatology

(2017) Rebekah M. Ahmed et al.   NEUROSCIENCE AND BIOBEHAVIORAL REVIEWS

The role of mitochondria in amyotrophic lateral sclerosis

(2017) Emma F. Smith et al.   NEUROSCIENCE LETTERS

DNA strand breaks and TDP-43 mislocation are absent in the murine hSOD1G93A model of amyotrophic lateral sclerosis in vivo and in vitro

(2017) Diane Penndorf et al.   PLoS One

Endoplasmic reticulum-mitochondria tethering in neurodegenerative diseases

(2017) Yi Liu et al.   Translational Neurodegeneration

Apical dendrite degeneration, a novel cellular pathology for Betz cells in ALS

(2017) Barış Genç et al.   Scientific Reports

Clinical Trials in a Dish: The Potential of Pluripotent Stem Cells to Develop Therapies for Neurodegenerative Diseases

(2016) Kelly M. Haston et al.   Annual Review of Pharmacology and Toxicology

Assessment of the upper motor neuron in amyotrophic lateral sclerosis

(2016) William Huynh et al.   CLINICAL NEUROPHYSIOLOGY

Current Therapy of Drugs in Amyotrophic Lateral Sclerosis

(2016) Haiyan Lu et al.   Current Neuropharmacology

Absence of alsin function leads to corticospinal motor neuron vulnerability via novel disease mechanisms

(2016) Mukesh Gautam et al.   HUMAN MOLECULAR GENETICS

Enhancing survival motor neuron expression extends lifespan and attenuates neurodegeneration in mutant TDP-43 mice

(2016) Nirma D. Perera et al.   HUMAN MOLECULAR GENETICS

Decoding ALS: from genes to mechanism

(2016) J. Paul Taylor et al.   NATURE

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

(2016) Wenzhang Wang et al.   NATURE MEDICINE

Pathophysiological and diagnostic implications of cortical dysfunction in ALS

(2016) Nimeshan Geevasinga et al.   Nature Reviews Neurology

Gamma motor neurons survive and exacerbate alpha motor neuron degeneration in ALS

(2016) Melanie Lalancette-Hebert et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

There's Something Wrong with my MAM; the ER–Mitochondria Axis and Neurodegenerative Diseases

(2016) Sebastien Paillusson et al.   TRENDS IN NEUROSCIENCES

ALS-linked misfolded SOD1 species have divergent impacts on mitochondria

(2016) Sarah Pickles et al.   Acta Neuropathologica Communications

Marked changes in dendritic structure and spine density precede significant neuronal death in vulnerable cortical pyramidal neuron populations in the SOD1G93A mouse model of amyotrophic lateral sclerosis

(2016) Matthew J. Fogarty et al.   Acta Neuropathologica Communications

TDP-43 or FUS-induced misfolded human wild-type SOD1 can propagate intercellularly in a prion-like fashion

(2016) Edward Pokrishevsky et al.   Scientific Reports

Cortical synaptic and dendritic spine abnormalities in a presymptomatic TDP-43 model of amyotrophic lateral sclerosis

(2016) Matthew J. Fogarty et al.   Scientific Reports

Corticospinal Motor Neurons Are Susceptible to Increased ER Stress and Display Profound Degeneration in the Absence of UCHL1 Function

(2015) Javier H. Jara et al.   CEREBRAL CORTEX

A Chemical Chaperone-Based Drug Candidate is Effective in a Mouse Model of Amyotrophic Lateral Sclerosis (ALS)

(2015) Tamar Getter et al.   ChemMedChem

Mitochondria in the Regulation of Innate and Adaptive Immunity

(2015) Samuel E. Weinberg et al.   IMMUNITY

Motor Cortex Layer V Pyramidal Neurons Exhibit Dendritic Regression, Spine Loss, and Increased Synaptic Excitation in the Presymptomatic hSOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis

(2015) M. J. Fogarty et al.   JOURNAL OF NEUROSCIENCE

Sensitivity and specificity of threshold tracking transcranial magnetic stimulation for diagnosis of amyotrophic lateral sclerosis: a prospective study

(2015) Parvathi Menon et al.   LANCET NEUROLOGY

An Assessment of Possible Neuropathology and Clinical Relationships in 46 Sporadic Amyotrophic Lateral Sclerosis Patient Autopsies

(2015) Grant Coan et al.   Neurodegenerative Diseases

Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants

(2015) Shahram Saberi et al.   NEUROLOGIC CLINICS

Edaravone, a Free Radical Scavenger, Delayed Symptomatic and Pathological Progression of Motor Neuron Disease in the Wobbler Mouse

(2015) Ken Ikeda et al.   PLoS One

SOD1 misplacing and mitochondrial dysfunction in amyotrophic lateral sclerosis pathogenesis

(2015) Francesco Tafuri et al.   Frontiers in Cellular Neuroscience

Golgi Fragmentation in ALS Motor Neurons. New Mechanisms Targeting Microtubules, Tethers, and Transport Vesicles

(2015) Georg Haase et al.   Frontiers in Neuroscience

Golgi fragmentation in amyotrophic lateral sclerosis, an overview of possible triggers and consequences

(2015) Vinod Sundaramoorthy et al.   Frontiers in Neuroscience

Potential Therapeutic Drugs and Methods for the Treatment of Amyotrophic Lateral Sclerosis

(2014) G. Yacila et al.   CURRENT MEDICINAL CHEMISTRY

The past, present and future of stem cell clinical trials for ALS

(2014) Gretchen M. Thomsen et al.   EXPERIMENTAL NEUROLOGY

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

Preclinical research: Make mouse studies work

(2014) Steve Perrin   NATURE

Early-Stage Treatment with Withaferin A Reduces Levels of Misfolded Superoxide Dismutase 1 and Extends Lifespan in a Mouse Model of Amyotrophic Lateral Sclerosis

(2014) Priyanka Patel et al.   Neurotherapeutics

Exome Sequencing Links Corticospinal Motor Neuron Disease to Common Neurodegenerative Disorders

(2014) G. Novarino et al.   SCIENCE

Treatment of Amyotrophic Lateral Sclerosis: Lessons Learned from Many Failures

(2014) P. Hande Özdinler et al.   ACS Medicinal Chemistry Letters

Retrograde labeling, transduction, and genetic targeting allow cellular analysis of corticospinal motor neurons: implications in health and disease

(2014) Javier H. Jara et al.   Frontiers in Neuroanatomy

Increased metal content in the TDP-43A315T transgenic mouse model of frontotemporal lobar degeneration and amyotrophic lateral sclerosis

(2014) Theresa N. T. Dang et al.   Frontiers in Aging Neuroscience

Hereditary spastic paraplegia: clinico-pathologic features and emerging molecular mechanisms

(2013) John K. Fink   ACTA NEUROPATHOLOGICA

Recent progress in the discovery of small molecules for the treatment of amyotrophic lateral sclerosis (ALS)

(2013) Allison S Limpert et al.   Beilstein Journal of Organic Chemistry

C57BL/6J congenic Prp-TDP43A315T mice develop progressive neurodegeneration in the myenteric plexus of the colon without exhibiting key features of ALS

(2013) Theo Hatzipetros et al.   BRAIN RESEARCH

Could Dysregulation of UPS be a Common Underlying Mechanism for Cancer and Neurodegeneration? Lessons from UCHL1

(2013) Javier H. Jara et al.   CELL BIOCHEMISTRY AND BIOPHYSICS

Moving forward in clinical trials for ALS: motor neurons lead the way please

(2013) Barış Genç et al.   DRUG DISCOVERY TODAY

Mitochondrial damage revealed by immunoselection for ALS-linked misfolded SOD1

(2013) S. Pickles et al.   HUMAN MOLECULAR GENETICS

Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders

(2013) J. Janssens et al.   HUMAN MOLECULAR GENETICS

eGFP Expression under UCHL1 Promoter Genetically Labels Corticospinal Motor Neurons and a Subpopulation of Degeneration-Resistant Spinal Motor Neurons in an ALS Mouse Model

(2013) M. V. Yasvoina et al.   JOURNAL OF NEUROSCIENCE

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

Of men, not mice

(2013)   NATURE MEDICINE

Molecular logic of neocortical projection neuron specification, development and diversity

(2013) Luciano Custo Greig et al.   NATURE REVIEWS NEUROSCIENCE

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

Calcium, mitochondria, and the pathogenesis of ALS: the good, the bad, and the ugly

(2013) Manoj Kumar Jaiswal   Frontiers in Cellular Neuroscience

Misfolded SOD1 and ALS: Zeroing in on mitochondria

(2012) Sarah Pickles et al.   Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis: New Insights into Underlying Molecular Mechanisms and Opportunities for Therapeutic Intervention

(2012) Mauro Cozzolino et al.   ANTIOXIDANTS & REDOX SIGNALING

Transcranial magnetic stimulation and amyotrophic lateral sclerosis: pathophysiological insights

(2012) Steve Vucic et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Corticospinal reorganization after spinal cord injury

(2012) Martin Oudega et al.   JOURNAL OF PHYSIOLOGY-LONDON

AAV2 mediated retrograde transduction of corticospinal motor neurons reveals initial and selective apical dendrite degeneration in ALS

(2012) Javier H. Jara et al.   NEUROBIOLOGY OF DISEASE

Chiral Cyclohexane 1,3-Diones as Inhibitors of Mutant SOD1-Dependent Protein Aggregation for the Treatment of ALS

(2012) Yinan Zhang et al.   ACS Medicinal Chemistry Letters

Cyclohexane 1,3-diones and their inhibition of mutant SOD1-dependent protein aggregation and toxicity in PC12 cells

(2011) Wei Zhang et al.   BIOORGANIC & MEDICINAL CHEMISTRY

Corticospinal Motor Neurons and Related Subcerebral Projection Neurons Undergo Early and Specific Neurodegeneration in hSOD1G93A Transgenic ALS Mice

(2011) P. H. Ozdinler et al.   JOURNAL OF NEUROSCIENCE

Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration

(2011) Edward B. Lee et al.   NATURE REVIEWS NEUROSCIENCE

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

(2011) Christine Vande Velde et al.   PLoS One

Mitochondrial dysfunction in ALS

(2011) Mauro Cozzolino et al.   PROGRESS IN NEUROBIOLOGY

Identification of compounds protective against G93A-SOD1 toxicity for the treatment of amyotrophic lateral sclerosis

(2010) Radhia Benmohamed et al.   Amyotrophic Lateral Sclerosis

Motor Neuron Diversity in Development and Disease

(2010) Kevin C. Kanning et al.   Annual Review of Neuroscience

A Review of the Neural Mechanisms of Action and Clinical Efficiency of Riluzole in Treating Amyotrophic Lateral Sclerosis: What have we Learned in the Last Decade?

(2010) Mark C. Bellingham   CNS Neuroscience & Therapeutics

The “Dying-Back” Phenomenon of Motor Neurons in ALS

(2010) Michal Dadon-Nachum et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Intracerebroventricular infusion of monoclonal antibody or its derived Fab fragment against misfolded forms of SOD1 mutant delays mortality in a mouse model of ALS

(2010) Francois Gros-Louis et al.   JOURNAL OF NEUROCHEMISTRY

Sublayer-specific microcircuits of corticospinal and corticostriatal neurons in motor cortex

(2010) Charles T Anderson et al.   NATURE NEUROSCIENCE

Defining the mechanisms that underlie cortical hyperexcitability in amyotrophic lateral sclerosis

(2009) Steve Vucic et al.   EXPERIMENTAL NEUROLOGY

Tests to assess motor phenotype in mice: a user's guide

(2009) Simon P. Brooks et al.   NATURE REVIEWS NEUROSCIENCE

TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations

(2009) Satomi Maekawa et al.   NEUROPATHOLOGY

TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration

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

Gamma and alpha motor neurons distinguished by expression of transcription factor Err3

(2009) A. Friese et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

(2008) Sean Scott et al.   Amyotrophic Lateral Sclerosis

TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy

(2008) Bradley J Turner et al.   BMC NEUROSCIENCE

Treatment with edaravone, initiated at symptom onset, slows motor decline and decreases SOD1 deposition in ALS mice

(2008) Hidefumi Ito et al.   EXPERIMENTAL NEUROLOGY

Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria

(2008) C. Vande Velde et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

EDARAVONE INHIBITS THE INDUCTION OF INOS GENE EXPRESSION AT TRANSCRIPTIONAL AND POSTTRANSCRIPTIONAL STEPS IN MURINE MACROPHAGES

(2008) Hideyuki Yoshida et al.   SHOCK

Edaravone prevents iNOS expression by inhibiting its promoter transactivation and mRNA stability in cytokine-stimulated hepatocytes

(2007) Hideyuki Yoshida et al.   NITRIC OXIDE-BIOLOGY AND CHEMISTRY