Nicholas J. Maragakis

Publications

• Mini-Review: Induced Pluripotent Stem Cells and the Search for New Cell-specific ALS Therapeutic Targets
  Neuroscience Letters. 06, 2021  |   Pubmed ID: 33892003
• Effect of Ezogabine on Cortical and Spinal Motor Neuron Excitability in Amyotrophic Lateral Sclerosis: A Randomized Clinical Trial
  JAMA Neurology. Feb, 2021  |   Pubmed ID: 33226425
• Phase 1-2 Trial of Antisense Oligonucleotide Tofersen for ALS
  The New England Journal of Medicine. 07, 2020  |   Pubmed ID: 32640130
• Amyotrophic Lateral Sclerosis Care and Research in the United States During the COVID-19 Pandemic: Challenges and Opportunities
  Muscle & Nerve. 08, 2020  |   Pubmed ID: 32445195
• Expression and Cellular Distribution of P-Glycoprotein and Breast Cancer Resistance Protein in Amyotrophic Lateral Sclerosis Patients
  Journal of Neuropathology and Experimental Neurology. 03, 2020  |   Pubmed ID: 31999342
• Persistent Cyfip1 Expression Is Required to Maintain the Adult Subventricular Zone Neurogenic Niche
  The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. 03, 2020  |   Pubmed ID: 31988061
• Addressing Heterogeneity in Amyotrophic Lateral Sclerosis CLINICAL TRIALS
  Muscle & Nerve. 08, 2020  |   Pubmed ID: 31899540
• Focal and Dose-dependent Neuroprotection in ALS Mice Following AAV2-neurturin Delivery
  Experimental Neurology. 01, 2020  |   Pubmed ID: 31678350
• Role of Human-Induced Pluripotent Stem Cell-Derived Spinal Cord Astrocytes in the Functional Maturation of Motor Neurons in a Multielectrode Array System
  Stem Cells Translational Medicine. 12, 2019  |   Pubmed ID: 31631575
• Perfluorocarbon Labeling of Human Glial-Restricted Progenitors for F Magnetic Resonance Imaging
  Stem Cells Translational Medicine. 04, 2019  |   Pubmed ID: 30618148
• Current and Emerging ALS Biomarkers: Utility and Potential in Clinical Trials
  Expert Review of Neurotherapeutics. Nov, 2018  |   Pubmed ID: 30273061
• Olfactory Dysfunction in Amyotrophic Lateral Sclerosis
  Annals of Clinical and Translational Neurology. Aug, 2018  |   Pubmed ID: 30128322
• Motor Neuron-derived MicroRNAs Cause Astrocyte Dysfunction in Amyotrophic Lateral Sclerosis
  Brain : a Journal of Neurology. 09, 2018  |   Pubmed ID: 30007309
• A Stocked Toolbox for Understanding the Role of Astrocytes in Disease
  Nature Reviews. Neurology. 06, 2018  |   Pubmed ID: 29769699
• Genome-wide Analyses Identify KIF5A As a Novel ALS Gene
  Neuron. Mar, 2018  |   Pubmed ID: 29566793
• A Randomized Controlled Trial of Resistance and Endurance Exercise in Amyotrophic Lateral Sclerosis
  Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. 05, 2018  |   Pubmed ID: 29191052
• Fibroblast Bioenergetics to Classify Amyotrophic Lateral Sclerosis Patients
  Molecular Neurodegeneration. 10, 2017  |   Pubmed ID: 29065921
• Erratum
  Advances in Neurobiology. 2017  |   Pubmed ID: 28905310
• What Can We Learn from the Edaravone Development Program for ALS?
  Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration. Oct, 2017  |   Pubmed ID: 28872911
• Motoneuron Disease: Clinical
  Advances in Neurobiology. 2017  |   Pubmed ID: 28674982
• Motoneuron Disease: Basic Science
  Advances in Neurobiology. 2017  |   Pubmed ID: 28674981
• MicroRNA Profiling Reveals Marker of Motor Neuron Disease in ALS Models
  The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. 05, 2017  |   Pubmed ID: 28416596
• Sporadic ALS Astrocytes Induce Neuronal Degeneration In Vivo
  Stem Cell Reports. 04, 2017  |   Pubmed ID: 28366455
• Serial in Vivo Imaging of Transplanted Allogeneic Neural Stem Cell Survival in a Mouse Model of Amyotrophic Lateral Sclerosis
  Experimental Neurology. 03, 2017  |   Pubmed ID: 28038988
• Defining SOD1 ALS Natural History to Guide Therapeutic Clinical Trial Design
  Journal of Neurology, Neurosurgery, and Psychiatry. Feb, 2017  |   Pubmed ID: 27261500
• 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
  Glia. 08, 2016  |   Pubmed ID: 27158936
• Connexin 43 in Astrocytes Contributes to Motor Neuron Toxicity in Amyotrophic Lateral Sclerosis
  Glia. 07, 2016  |   Pubmed ID: 27083773
• Primary Lateral Sclerosis and Early Upper Motor Neuron Disease: Characteristics of a Cross-Sectional Population
  Journal of Clinical Neuromuscular Disease. Mar, 2016  |   Pubmed ID: 26905909
• Generation of GFAP::GFP Astrocyte Reporter Lines from Human Adult Fibroblast-derived IPS Cells Using Zinc-finger Nuclease Technology
  Glia. Jan, 2016  |   Pubmed ID: 26295203
• Hemiparetic Primary Lateral Sclerosis: Revisiting Mills Syndrome
  Case Reports in Neurology. Sep-Dec, 2015  |   Pubmed ID: 26500542
• Human IPS Cell-derived Astrocyte Transplants Preserve Respiratory Function After Spinal Cord Injury
  Experimental Neurology. Sep, 2015  |   Pubmed ID: 26216662
• Neural and Glial Progenitor Transplantation As a Neuroprotective Strategy for Amyotrophic Lateral Sclerosis (ALS)
  Brain Research. Dec, 2015  |   Pubmed ID: 26187754
• A Comprehensive Library of Familial Human Amyotrophic Lateral Sclerosis Induced Pluripotent Stem Cells
  PloS One. 2015  |   Pubmed ID: 25760436
• Peripheral Neuropathy in a Family with Sandhoff Disease and SH3TC2 Deficiency
  Journal of Neurology. 2015  |   Pubmed ID: 25736553
• Human Glial Progenitor Engraftment and Gene Expression is Independent of the ALS Environment
  Experimental Neurology. Feb, 2015  |   Pubmed ID: 25523812
• Transplantation of Glial Progenitors That Overexpress Glutamate Transporter GLT1 Preserves Diaphragm Function Following Cervical SCI
  Molecular Therapy : the Journal of the American Society of Gene Therapy. Mar, 2015  |   Pubmed ID: 25492561
• Induced Pluripotent Stem Cells from ALS Patients for Disease Modeling
  Brain Research. May, 2015  |   Pubmed ID: 25223906
• Gene Profiling of Human Induced Pluripotent Stem Cell-derived Astrocyte Progenitors Following Spinal Cord Engraftment
  Stem Cells Translational Medicine. May, 2014  |   Pubmed ID: 24604284
• C9orf72 Nucleotide Repeat Structures Initiate Molecular Cascades of Disease
  Nature. Mar, 2014  |   Pubmed ID: 24598541
• Altered Astrocytic Expression of TDP-43 Does Not Influence Motor Neuron Survival
  Experimental Neurology. Dec, 2013  |   Pubmed ID: 24120466
• Exome Sequencing to Identify De Novo Mutations in Sporadic ALS Trios
  Nature Neuroscience. Jul, 2013  |   Pubmed ID: 23708140
• Glia: an Emerging Target for Neurological Disease Therapy
  Stem Cell Research & Therapy. Sep, 2012  |   Pubmed ID: 23021042
• Creation of an Open-access, Mutation-defined Fibroblast Resource for Neurological Disease Research
  PloS One. 2012  |   Pubmed ID: 22952635
• Selective Increase of Two ABC Drug Efflux Transporters at the Blood-spinal Cord Barrier Suggests Induced Pharmacoresistance in ALS
  Neurobiology of Disease. Aug, 2012  |   Pubmed ID: 22521463
• Human Glial-restricted Progenitor Transplantation into Cervical Spinal Cord of the SOD1 Mouse Model of ALS
  PloS One. 2011  |   Pubmed ID: 21998733
• Astrocytes Carrying the Superoxide Dismutase 1 (SOD1G93A) Mutation Induce Wild-type Motor Neuron Degeneration in Vivo
  Proceedings of the National Academy of Sciences of the United States of America. Oct, 2011  |   Pubmed ID: 21969586
• Stem Cell Transplantation for Spinal Cord Neurodegeneration
  Methods in Molecular Biology (Clifton, N.J.). 2011  |   Pubmed ID: 21913120
• Reduction in Expression of the Astrocyte Glutamate Transporter, GLT1, Worsens Functional and Histological Outcomes Following Traumatic Spinal Cord Injury
  Glia. Aug, 2011  |   Pubmed ID: 21882244
• Spatial and Temporal Changes in Promoter Activity of the Astrocyte Glutamate Transporter GLT1 Following Traumatic Spinal Cord Injury
  Journal of Neuroscience Research. Jul, 2011  |   Pubmed ID: 21488085
• Stem Cells and the ALS Neurologist
  Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases. Oct, 2010  |   Pubmed ID: 20509745
• Peripheral Hyperstimulation Alters Site of Disease Onset and Course in SOD1 Rats
  Neurobiology of Disease. Sep, 2010  |   Pubmed ID: 20381620
• Advances in Stem Cell Research for Amyotrophic Lateral Sclerosis
  Current Opinion in Biotechnology. Oct, 2009  |   Pubmed ID: 19819686
• A Targeted Neuroglial Reporter Line Generated by Homologous Recombination in Human Embryonic Stem Cells
  Stem Cells (Dayton, Ohio). Aug, 2009  |   Pubmed ID: 19544414
• Rethinking a Drug Treatment Failure on a Traditional ALS Target
  Experimental Neurology. Apr, 2009  |   Pubmed ID: 19309797
• Accurate ALSFRS-R Scores Can Be Generated from Retrospective Review of Clinic Notes
  Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases. Aug, 2009  |   Pubmed ID: 18985465
• Focal Transplantation-based Astrocyte Replacement is Neuroprotective in a Model of Motor Neuron Disease
  Nature Neuroscience. Nov, 2008  |   Pubmed ID: 18931666
• Selective Ablation of Proliferating Astrocytes Does Not Affect Disease Outcome in Either Acute or Chronic Models of Motor Neuron Degeneration
  Experimental Neurology. Jun, 2008  |   Pubmed ID: 18410928
• Intraparenchymal Spinal Cord Delivery of Adeno-associated Virus IGF-1 is Protective in the SOD1G93A Model of ALS
  Brain Research. Dec, 2007  |   Pubmed ID: 17963733
• Mechanisms of Disease: Astrocytes in Neurodegenerative Disease
  Nature Clinical Practice. Neurology. Dec, 2006  |   Pubmed ID: 17117171
• Loss of the Astrocyte Glutamate Transporter GLT1 Modifies Disease in SOD1(G93A) Mice
  Experimental Neurology. Sep, 2006  |   Pubmed ID: 16753145
• Selective Up-regulation of the Glial Na+-dependent Glutamate Transporter GLT1 by a Neuroimmunophilin Ligand Results in Neuroprotection
  Neurobiology of Disease. Mar, 2006  |   Pubmed ID: 16274998
• Degeneration of Respiratory Motor Neurons in the SOD1 G93A Transgenic Rat Model of ALS
  Neurobiology of Disease. Jan, 2006  |   Pubmed ID: 16084734
• Glial Restricted Precursors Protect Against Chronic Glutamate Neurotoxicity of Motor Neurons in Vitro
  Glia. Apr, 2005  |   Pubmed ID: 15657939
• Neural Stem Cells Protect Against Glutamate-induced Excitotoxicity and Promote Survival of Injured Motor Neurons Through the Secretion of Neurotrophic Factors
  Molecular and Cellular Neurosciences. Nov, 2004  |   Pubmed ID: 15519246
• Viral-induced Spinal Motor Neuron Death is Non-cell-autonomous and Involves Glutamate Excitotoxicity
  The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Aug, 2004  |   Pubmed ID: 15329404
• Glutamate Transporters: Animal Models to Neurologic Disease
  Neurobiology of Disease. Apr, 2004  |   Pubmed ID: 15056453
• Altered Expression of the Glutamate Transporter EAAT2b in Neurological Disease
  Annals of Neurology. Apr, 2004  |   Pubmed ID: 15048885
• Glutamate Transporter Expression and Function in Human Glial Progenitors
  Glia. Jan, 2004  |   Pubmed ID: 14730707
• Human Embryonic Germ Cell Derivatives Facilitate Motor Recovery of Rats with Diffuse Motor Neuron Injury
  The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Jun, 2003  |   Pubmed ID: 12832537
• Topiramate Protects Against Motor Neuron Degeneration in Organotypic Spinal Cord Cultures but Not in G93A SOD1 Transgenic Mice
  Neuroscience Letters. Feb, 2003  |   Pubmed ID: 12566164
• Amyotrophic Lateral Sclerosis: Pathogenesis, Differential Diagnoses, and Potential Interventions
  The Journal of Spinal Cord Medicine. 2002  |   Pubmed ID: 12482167
• Focal Loss of the Glutamate Transporter EAAT2 in a Transgenic Rat Model of SOD1 Mutant-mediated Amyotrophic Lateral Sclerosis (ALS)
  Proceedings of the National Academy of Sciences of the United States of America. Feb, 2002  |   Pubmed ID: 11818550