Nicholas J. Maragakis

Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS).

Amyotrophic lateral sclerosis: pathogenesis, differential diagnoses, and potential interventions.

Topiramate protects against motor neuron degeneration in organotypic spinal cord cultures but not in G93A SOD1 transgenic mice.

Human embryonic germ cell derivatives facilitate motor recovery of rats with diffuse motor neuron injury.

Glutamate transporter expression and function in human glial progenitors.

Altered expression of the glutamate transporter EAAT2b in neurological disease.

Glutamate transporters: animal models to neurologic disease.

Viral-induced spinal motor neuron death is non-cell-autonomous and involves glutamate excitotoxicity.

Neural stem cells protect against glutamate-induced excitotoxicity and promote survival of injured motor neurons through the secretion of neurotrophic factors.

Glial restricted precursors protect against chronic glutamate neurotoxicity of motor neurons in vitro.

Degeneration of respiratory motor neurons in the SOD1 G93A transgenic rat model of ALS.

Selective up-regulation of the glial Na+-dependent glutamate transporter GLT1 by a neuroimmunophilin ligand results in neuroprotection.

Loss of the astrocyte glutamate transporter GLT1 modifies disease in SOD1(G93A) mice.

Mechanisms of Disease: astrocytes in neurodegenerative disease.

Intraparenchymal spinal cord delivery of adeno-associated virus IGF-1 is protective in the SOD1G93A model of ALS.

Selective ablation of proliferating astrocytes does not affect disease outcome in either acute or chronic models of motor neuron degeneration.

Focal transplantation-based astrocyte replacement is neuroprotective in a model of motor neuron disease.

Accurate ALSFRS-R scores can be generated from retrospective review of clinic notes.

Rethinking a drug treatment failure on a traditional ALS target.

A targeted neuroglial reporter line generated by homologous recombination in human embryonic stem cells.

Advances in stem cell research for Amyotrophic Lateral Sclerosis.

Peripheral hyperstimulation alters site of disease onset and course in SOD1 rats.

Stem cells and the ALS neurologist.

Spatial and temporal changes in promoter activity of the astrocyte glutamate transporter GLT1 following traumatic spinal cord injury.

Reduction in expression of the astrocyte glutamate transporter, GLT1, worsens functional and histological outcomes following traumatic spinal cord injury.

Stem cell transplantation for spinal cord neurodegeneration.

Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo.

Human glial-restricted progenitor transplantation into cervical spinal cord of the SOD1 mouse model of ALS.

Selective increase of two ABC drug efflux transporters at the blood-spinal cord barrier suggests induced pharmacoresistance in ALS.

Creation of an open-access, mutation-defined fibroblast resource for neurological disease research.

Glia: an emerging target for neurological disease therapy.

Exome sequencing to identify de novo mutations in sporadic ALS trios.

Altered astrocytic expression of TDP-43 does not influence motor neuron survival.

C9orf72 nucleotide repeat structures initiate molecular cascades of disease.

Gene profiling of human induced pluripotent stem cell-derived astrocyte progenitors following spinal cord engraftment.

Induced pluripotent stem cells from ALS patients for disease modeling.

Transplantation of glial progenitors that overexpress glutamate transporter GLT1 preserves diaphragm function following cervical SCI.

Human glial progenitor engraftment and gene expression is independent of the ALS environment.

Peripheral neuropathy in a family with Sandhoff disease and SH3TC2 deficiency.

A comprehensive library of familial human amyotrophic lateral sclerosis induced pluripotent stem cells.

Neural and glial progenitor transplantation as a neuroprotective strategy for Amyotrophic Lateral Sclerosis (ALS).

Human iPS cell-derived astrocyte transplants preserve respiratory function after spinal cord injury.

Generation of GFAP::GFP astrocyte reporter lines from human adult fibroblast-derived iPS cells using zinc-finger nuclease technology.

Hemiparetic Primary Lateral Sclerosis: Revisiting Mills Syndrome.

Primary Lateral Sclerosis and Early Upper Motor Neuron Disease: Characteristics of a Cross-Sectional Population.

Connexin 43 in astrocytes contributes to motor neuron toxicity in amyotrophic lateral sclerosis.

Astrocytes drive upregulation of the multidrug resistance transporter ABCB1 (P-Glycoprotein) in endothelial cells of the blood-brain barrier in mutant superoxide dismutase 1-linked amyotrophic lateral sclerosis.

Defining SOD1 ALS natural history to guide therapeutic clinical trial design.

Serial in vivo imaging of transplanted allogeneic neural stem cell survival in a mouse model of amyotrophic lateral sclerosis.

Sporadic ALS Astrocytes Induce Neuronal Degeneration In Vivo.

MicroRNA Profiling Reveals Marker of Motor Neuron Disease in ALS Models.

Motoneuron Disease: Basic Science.

Motoneuron Disease: Clinical.

What can we learn from the edaravone development program for ALS?

Erratum.

Fibroblast bioenergetics to classify amyotrophic lateral sclerosis patients.

A randomized controlled trial of resistance and endurance exercise in amyotrophic lateral sclerosis.

Genome-wide Analyses Identify KIF5A as a Novel ALS Gene.

A stocked toolbox for understanding the role of astrocytes in disease.

Motor neuron-derived microRNAs cause astrocyte dysfunction in amyotrophic lateral sclerosis.

Olfactory dysfunction in amyotrophic lateral sclerosis.

Current and emerging ALS biomarkers: utility and potential in clinical trials.

Perfluorocarbon Labeling of Human Glial-Restricted Progenitors for F Magnetic Resonance Imaging.

Role of Human-Induced Pluripotent Stem Cell-Derived Spinal Cord Astrocytes in the Functional Maturation of Motor Neurons in a Multielectrode Array System.

Focal and dose-dependent neuroprotection in ALS mice following AAV2-neurturin delivery.

Addressing heterogeneity in amyotrophic lateral sclerosis CLINICAL TRIALS.

Persistent Cyfip1 Expression Is Required to Maintain the Adult Subventricular Zone Neurogenic Niche.

Expression and Cellular Distribution of P-Glycoprotein and Breast Cancer Resistance Protein in Amyotrophic Lateral Sclerosis Patients.

Amyotrophic lateral sclerosis care and research in the United States during the COVID-19 pandemic: Challenges and opportunities.

Phase 1-2 Trial of Antisense Oligonucleotide Tofersen for ALS.

Effect of Ezogabine on Cortical and Spinal Motor Neuron Excitability in Amyotrophic Lateral Sclerosis: A Randomized Clinical Trial.

Mini-Review: Induced pluripotent stem cells and the search for new cell-specific ALS therapeutic targets.