Molecular pathways of motor neuron injury in 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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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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Onset and Progression in Inherited ALS Determined by Motor Neurons and Microglia

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

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

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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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Orchestrating the unfolded protein response in health and disease

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Selective neuronal vulnerability to oxidative stress in the brain

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

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

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

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

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

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Molecular mechanisms of calcium-dependent neurodegeneration in excitotoxicity

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

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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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Role of Axonal Transport in Neurodegenerative Diseases

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

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

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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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A role for motoneuron subtype–selective ER stress in disease manifestations of FALS mice

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Presence of dendritic cells, MCP‐1, and activated microglia/macrophages in amyotrophic lateral sclerosis spinal cord 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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Toxicity of Familial ALS-Linked SOD1 Mutants from Selective Recruitment to Spinal Mitochondria

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

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

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

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Non-Cell-Autonomous Effect of Human SOD1G37R Astrocytes on Motor Neurons Derived from Human Embryonic Stem Cells

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

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Deficits in axonal transport precede ALS symptoms in vivo

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Atomic structure and chemistry of human serum albumin

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

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ALS phenotypes with mutations in <i>CHMP2B</i> (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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Neuronal Death in Amyotrophic Lateral Sclerosis Is Apoptosis

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

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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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Glutamate Transporters and the Excitotoxic Path to Motor Neuron Degeneration in Amyotrophic Lateral Sclerosis

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Parvalbumin and calbindin D‐28k in the human motor system and in motor neuron disease

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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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Untitled work

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Serum miRNAs miR-206, 143-3p and 374b-5p as potential biomarkers for amyotrophic lateral sclerosis (ALS)

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Retroviruses and amyotrophic lateral sclerosis

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

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TDP-43/FUS in motor neuron disease: Complexity and challenges

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Amyotrophic lateral sclerosis-associated mutant VAPBP56S perturbs calcium homeostasis to disrupt axonal transport of mitochondria

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Expression of wild-type human superoxide dismutase-1 in mice causes amyotrophic lateral sclerosis

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Fused in sarcoma (FUS): An oncogene goes awry in neurodegeneration

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

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Mice deficient in Epg5 exhibit selective neuronal vulnerability to degeneration

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Massive transcriptome sequencing of human spinal cord tissues provides new insights into motor neuron degeneration in ALS

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The role of heat shock proteins in Amyotrophic Lateral Sclerosis: The therapeutic potential of Arimoclomol

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Synergistic Effects of GDNF and VEGF on Lifespan and Disease Progression in a Familial ALS Rat Model

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Neurotrophic Natural Products: Chemistry and Biology

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CNS glucose metabolism in Amyotrophic Lateral Sclerosis: a therapeutic target?

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Rho kinase inhibition modulates microglia activation and improves survival in a model of amyotrophic lateral sclerosis

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Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALS

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ALS‐linked protein disulfide isomerase variants cause motor dysfunction

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The ER mitochondria calcium cycle and ER stress response as therapeutic targets in amyotrophic lateral sclerosis

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

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Astrocytes expressing mutant SOD1 and TDP43 trigger motoneuron death that is mediated via sodium channels and nitroxidative stress

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Activation of mitochondrial transient receptor potential vanilloid 1 channel contributes to microglial migration

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Minimally invasive transplantation of iPSC-derived ALDHhiSSCloVLA4+ neural stem cells effectively improves the phenotype of an amyotrophic lateral sclerosis model

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Cannabinoid pharmacology/therapeutics in chronic degenerative disorders affecting the central nervous system

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Ablation of P2X7 receptor exacerbates gliosis and motoneuron death in the SOD1-G93A mouse model of amyotrophic lateral sclerosis

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Amyotrophic lateral sclerosis-associated mutant SOD1 inhibits anterograde axonal transport of mitochondria by reducing Miro1 levels

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Risk factors and emerging therapies in amyotrophic lateral sclerosis

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The distinct genetic pattern of ALS in Turkey and novel mutations

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Astrocyte adenosine deaminase loss increases motor neuron toxicity in amyotrophic lateral sclerosis

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Intermittent Hypoxia and Stem Cell Implants Preserve Breathing Capacity in a Rodent Model of Amyotrophic Lateral Sclerosis

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Ferroptosis regulation through Nrf2 and implications for neurodegenerative diseases

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Innate Immunity: A Common Denominator between Neurodegenerative and Neuropsychiatric Diseases

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Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?

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Structural and molecular insights into the mechanism of action of human angiogenin-ALS variants in neurons

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The wobbler mouse, an ALS animal model

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Reactive oxygen species trigger motoneuron death in non-cell-autonomous models of ALS through activation of c-Abl signaling

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Gene Therapy for ALS—A Perspective

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The cannabinoid system and microglia in health and disease

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Optineurin in amyotrophic lateral sclerosis: Multifunctional adaptor protein at the crossroads of different neuroprotective mechanisms

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

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Genetic heterogeneity of amyotrophic lateral sclerosis: Implications for clinical practice and research

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Early interneuron dysfunction in ALS: Insights from a mutant sod1 zebrafish model

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Cause or Effect: Misregulation of microRNA Pathways in Neurodegeneration

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SIRT1 overexpression ameliorates a mouse model of SOD1-linked amyotrophic lateral sclerosis via HSF1/HSP70i chaperone system

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C9orf72 expansion within astrocytes reduces metabolic flexibility in amyotrophic lateral sclerosis

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Nanocarrier-mediated brain delivery of bioactives for treatment/prevention of neurodegenerative diseases

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Neurodegeneration in C. elegans models of ALS requires TIR-1/Sarm1 immune pathway activation in neurons

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The multifaceted role of glial cells in amyotrophic lateral sclerosis

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Superoxide dismutase 1 mutation in a cellular model of amyotrophic lateral sclerosis shifts energy generation from oxidative phosphorylation to glycolysis

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Mitochondrial Dysfunction in Neurodegenerative Diseases

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Neuron-specific antioxidant OXR1 extends survival of a mouse model of amyotrophic lateral sclerosis

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Proteostasis impairment in ALS

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Chapter 4 Molecular mechanisms of motor neuron degeneration in amyotrophic lateral sclerosis

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