Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

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Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis

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

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Identification and characterization of a spinal muscular atrophy-determining gene

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Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17

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TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis

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

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

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An Immunohistochemical Study

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Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia

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Selective loss of glial glutamate transporter GLT‐1 in amyotrophic lateral sclerosis

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An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria

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Greenfield's Neuropathology

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Amyotrophic lateral sclerosis is a distal axonopathy: evidence in mice and man

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

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Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43

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Dose-ranging study of riluzole in amyotrophic lateral sclerosis

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Mutations of optineurin in amyotrophic lateral sclerosis

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Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

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Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons

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Decreased Glutamate Transport by the Brain and Spinal Cord in Amyotrophic Lateral Sclerosis

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

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Characterizing the RNA targets and position-dependent splicing regulation by TDP-43

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Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis

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Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice

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Selective neuronal vulnerability to oxidative stress in the brain

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Orchestrating the unfolded protein response in health and disease

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

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Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

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Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis

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A Mutation in the Vesicle-Trafficking Protein VAPB Causes Late-Onset Spinal Muscular Atrophy and Amyotrophic Lateral Sclerosis

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Molecular mechanisms of calcium-dependent neurodegeneration in excitotoxicity

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Involvement of caspase-4 in endoplasmic reticulum stress-induced apoptosis and Aβ-induced cell death

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ALS‐associated fused in sarcoma (FUS) mutations disrupt Transportin‐mediated nuclear import

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DNA/RNA Helicase Gene Mutations in a Form of Juvenile Amyotrophic Lateral Sclerosis (ALS4)

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Role of Axonal Transport in Neurodegenerative Diseases

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The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis

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Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?

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Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF

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Clinical genetics of amyotrophic lateral sclerosis: what do we really know?

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Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

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ANG mutations segregate with familial and 'sporadic' amyotrophic lateral sclerosis

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Slowing of axonal transport is a very early event in the toxicity of ALS–linked SOD1 mutants to motor neurons

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Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target

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Mutated Human SOD1 Causes Dysfunction of Oxidative Phosphorylation in Mitochondria of Transgenic Mice

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Tar DNA Binding Protein-43 (TDP-43) Associates with Stress Granules: Analysis of Cultured Cells and Pathological Brain Tissue

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Energy metabolism in amyotrophic lateral sclerosis

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XBP-1 deficiency in the nervous system protects against amyotrophic lateral sclerosis by increasing autophagy

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Axonal mitochondrial transport and potential are correlated

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Amyotrophic Lateral Sclerosis-Associated SOD1 Mutant Proteins Bind and Aggregate with Bcl-2 in Spinal Cord Mitochondria

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Human Embryonic Stem Cell-Derived Motor Neurons Are Sensitive to the Toxic Effect of Glial Cells Carrying an ALS-Causing Mutation

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CD4+ T cells support glial neuroprotection, slow disease progression, and modify glial morphology in an animal model of inherited ALS

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Variants of the heavy neurofilament subunit are associated with the development of amyotrophic lateral sclerosis

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Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis

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Identification of Neuronal RNA Targets of TDP-43-containing Ribonucleoprotein Complexes

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Motor Neurons Are Selectively Vulnerable to AMPA/Kainate Receptor-Mediated InjuryIn Vitro

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Deficits in axonal transport precede ALS symptoms in vivo

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

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Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content

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

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ALS phenotypes with mutations in CHMP2B (charged multivesicular body protein 2B)

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Restricted Expression of G86R Cu/Zn Superoxide Dismutase in Astrocytes Results in Astrocytosis But Does Not Cause Motoneuron Degeneration

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Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients

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

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Neuronal Death in Amyotrophic Lateral Sclerosis Is Apoptosis

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Lost in translation: Treatment trials in the SOD1 mouse and in human ALS

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Novel threshold tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron disease

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Increased lipid peroxidation in sera of ALS patients

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Identification of active loci of a human endogenous retrovirus in neurons of patients with amyotrophic lateral sclerosis

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Linkage of Familial Amyotrophic Lateral Sclerosis With Frontotemporal Dementia to Chromosome 9q21-q22

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

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Calcium dysregulation in amyotrophic lateral sclerosis

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Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis

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SPATACSIN mutations cause autosomal recessive juvenile amyotrophic lateral sclerosis

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TDP-43 Mediates Degeneration in a Novel Drosophila Model of Disease Caused by Mutations in VCP/p97

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