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 Etablering av en elektrofysiologisk plattform for modellering av ALS med regionalt spesifikke humane pluripotente stamcelleavledede astrocytter og nevroner 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
Etablering av en elektrofysiologisk plattform for modellering av ALS med regionalt spesifikke humane pluripotente stamcelleavledede astrocytter og nevroner 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