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 Humanos Glial Restringido Progenitor Trasplante En Médula Espinal Cervical Del Modelo De Ratón SOD1 De La ELA PloS One. 2011 | Pubmed ID: 21998733 Astrocitos Que La Superóxido Dismutasa 1 (SOD1G93A) Mutación Provocar El Tipo Salvaje Degeneración De Neuronas Motoras En Vivo Proceedings of the National Academy of Sciences of the United States of America. Oct, 2011 | Pubmed ID: 21969586 Trasplante De Células Madre Para La Neurodegeneración De La Médula Espinal Methods in Molecular Biology (Clifton, N.J.). 2011 | Pubmed ID: 21913120 Reducción En La Expresión Del Transportador De Glutamato Astrocitos, GLT1, Empeora Los Resultados Funcionales E Histológicas Después De Lesión Medular Traumática Glia. Aug, 2011 | Pubmed ID: 21882244 Cambios Espaciales Y Temporales En La Actividad Promotora Del Transportador De Glutamato GLT1 Astrocitos Después De Lesión Medular Traumática 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 Hiperestimulación Periférica Altera Sitio De La Aparición De Enfermedades Y Curso De SOD1 Ratas 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 Trasplante Focal Basada En Reemplazo De Los Astrocitos Es Neuroprotector En Un Modelo De Enfermedad De La Motoneurona Nature Neuroscience. Nov, 2008 | Pubmed ID: 18931666 La Ablación Selectiva De Los Astrocitos Proliferantes No Afecta El Resultado Enfermedad En Modelos Aguda O Crónica De La Degeneración De Neuronas Motoras Experimental Neurology. Jun, 2008 | Pubmed ID: 18410928 Entrega Intraparenquimatosa De La Médula Espinal De Los Virus Adeno-asociado De IGF-1 Tiene Un Efecto Protector En El Modelo De SOD1G93A De La ELA Brain Research. Dec, 2007 | Pubmed ID: 17963733 Los Mecanismos De La Enfermedad: Los Astrocitos En Las Enfermedades Neurodegenerativas Nature Clinical Practice. Neurology. Dec, 2006 | Pubmed ID: 17117171 La Pérdida Del Transportador De Glutamato GLT1 Astrocitos Modifica La Enfermedad En SOD1 G93A () Ratones Experimental Neurology. Sep, 2006 | Pubmed ID: 16753145 Selectivo Sobre Regulación De La Glial Na +-dependiente GLT1 Transportador De Glutamato Por Un Ligando Resultados Neuroimmunophilin En La Neuroprotección Neurobiology of Disease. Mar, 2006 | Pubmed ID: 16274998 La Degeneración De Las Neuronas Motoras Respiratorias En El Modelo De SOD1 G93A Rata Transgénica De La ELA Neurobiology of Disease. Jan, 2006 | Pubmed ID: 16084734 Los Precursores Gliales Restringidas Proteger Contra La Neurotoxicidad Del Glutamato Crónica De Neuronas Motoras En Vitro Glia. Apr, 2005 | Pubmed ID: 15657939 Las Células Troncales Nerviosas Proteger Contra El Glutamato Inducida Por Excitotoxicidad Y Promover La Supervivencia De Las Neuronas Motoras Heridos Por La Secreción De Factores Neurotróficos Molecular and Cellular Neurosciences. Nov, 2004 | Pubmed ID: 15519246 Viral Inducida Por La Columna Vertebral Del Motor Es La Muerte De Neuronas No Células Autónomas E Implica La Excitotoxicidad Del Glutamato The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Aug, 2004 | Pubmed ID: 15329404 Los Transportadores De Glutamato: Modelos Animales Para Enfermedades Neurológicas Neurobiology of Disease. Apr, 2004 | Pubmed ID: 15056453 La Expresión Alterada De La EAAT2b Transportador De Glutamato En La Enfermedad Neurológica Annals of Neurology. Apr, 2004 | Pubmed ID: 15048885 Transportador De Glutamato Expresión Y Función En Humanos Progenitores Gliales Glia. Jan, 2004 | Pubmed ID: 14730707 Humanos Derivados De Células Germinales Embrionarias Facilitar La Recuperación Motora De Las Ratas Con Lesión Difusa Del Motor Neuronal The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Jun, 2003 | Pubmed ID: 12832537 El Topiramato Protege Contra La Degeneración De Neuronas Motoras En La Médula Espinal Organotípicos Culturas, Pero No En SOD1 G93A Ratones Transgénicos 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 Pérdida Focal De La EAAT2 Transportador De Glutamato En Un Modelo De Rata Transgénica De La SOD1 Mutante Mediado Por La Esclerosis Lateral Amiotrófica (ELA) Proceedings of the National Academy of Sciences of the United States of America. Feb, 2002 | Pubmed ID: 11818550 Establecimiento de una plataforma electrofisiológica para modelar la ELA con astrocitos y neuronas derivados de células madre pluripotentes humanas específicas de la región 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 신경과학
Establecimiento de una plataforma electrofisiológica para modelar la ELA con astrocitos y neuronas derivados de células madre pluripotentes humanas específicas de la región 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 신경과학