Nicholas J. Maragakis Department of Neurology Johns Hopkins University Biography Publications Institution JoVE Articles Nicholas J. Maragakis has not added a biography. If you are Nicholas J. Maragakis and would like to personalize this page please email our Author Liaison for assistance. 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 Establishment of an Electrophysiological Platform for Modeling ALS with Regionally-Specific Human Pluripotent Stem Cell-Derived Astrocytes and Neurons Arens Taga*1, Christa W. Habela*1, Alexandra Johns1, Shiyu Liu1, Mollie O'Brien1, Nicholas J. Maragakis1 1Department of Neurology, Johns Hopkins University JoVE 62726 Neuroscience
Establishment of an Electrophysiological Platform for Modeling ALS with Regionally-Specific Human Pluripotent Stem Cell-Derived Astrocytes and Neurons Arens Taga*1, Christa W. Habela*1, Alexandra Johns1, Shiyu Liu1, Mollie O'Brien1, Nicholas J. Maragakis1 1Department of Neurology, Johns Hopkins University JoVE 62726 Neuroscience