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 人类神经胶质限制祖移植 SOD1 小鼠模型的 ALS 颈脊髓损伤。 PloS One. 2011 | Pubmed ID: 21998733 携带 1 (SOD1G93A) 超氧化物歧化酶的星形胶质细胞突变诱使野生型运动神经元退行性变的体内。 Proceedings of the National Academy of Sciences of the United States of America. Oct, 2011 | Pubmed ID: 21969586 干细胞移植治疗脊髓神经变性。 Methods in Molecular Biology (Clifton, N.J.). 2011 | Pubmed ID: 21913120 在表达式中的星形胶质细胞谷氨酸转运体,GLT1,减少恶化外伤性脊髓损伤后的功能和组织学的成果。 Glia. Aug, 2011 | Pubmed ID: 21882244 启动子活性的星形胶质细胞谷氨酸转运体 GLT1 外伤性脊髓损伤后的空间和时间变化。 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 外围过度刺激改变疾病发生和发展过程中 SOD1 大鼠的站点。 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 焦基于移植的星形胶质细胞替换是在模型中的运动神经元病的神经保护作用。 Nature Neuroscience. Nov, 2008 | Pubmed ID: 18931666 细胞核星形胶质细胞的选择性消融并不影响疾病急性或慢性模型的运动神经元退行性变的结果。 Experimental Neurology. Jun, 2008 | Pubmed ID: 18410928 Intraparenchymal 脊髓传递的腺相关病毒胰岛素样生长因子-1 是防护的 ALS SOD1G93A 模型中。 Brain Research. Dec, 2007 | Pubmed ID: 17963733 疾病的机制: 星形胶质细胞神经退行性疾病。 Nature Clinical Practice. Neurology. Dec, 2006 | Pubmed ID: 17117171 损失的星形胶质细胞谷氨酸转运体 GLT1 修改 SOD1(G93A) 小鼠的疾病。 Experimental Neurology. Sep, 2006 | Pubmed ID: 16753145 选择性调控胶质细胞 Na +-依赖谷氨酸转运 GLT1 Neuroimmunophilin 配体的神经保护作用的结果。 Neurobiology of Disease. Mar, 2006 | Pubmed ID: 16274998 ALS SOD1 G93A 转基因大鼠模型中的呼吸电机神经元变性。 Neurobiology of Disease. Jan, 2006 | Pubmed ID: 16084734 针对慢性谷氨酸神经毒性的体外运动神经元保护胶质限制的前体。 Glia. Apr, 2005 | Pubmed ID: 15657939 神经干细胞兴奋性对保护和促进受损伤运动神经元通过分泌神经营养因子的生存。 Molecular and Cellular Neurosciences. Nov, 2004 | Pubmed ID: 15519246 病毒诱导脊髓运动神经元死亡非自治的单元格,包括谷氨酸兴奋毒性。 The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Aug, 2004 | Pubmed ID: 15329404 谷氨酸转运体: 对神经系统疾病的动物模型。 Neurobiology of Disease. Apr, 2004 | Pubmed ID: 15056453 谷氨酸转运蛋白 EAAT2b 在神经系统疾病中的表达变化。 Annals of Neurology. Apr, 2004 | Pubmed ID: 15048885 谷氨酸转运体表达和人类神经胶质祖细胞的功能。 Glia. Jan, 2004 | Pubmed ID: 14730707 人类胚胎生殖细胞衍生品便利弥漫性运动神经元损伤大鼠运动功能恢复。 The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Jun, 2003 | Pubmed ID: 12832537 托吡酯可防止运动神经元退化器官脊髓文化中但不是在 G93A SOD1 转基因小鼠。 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 SOD1 突变体介导萎缩侧索硬化 (ALS) 转基因大鼠模型中的谷氨酸转运体 EAAT2 焦损失。 Proceedings of the National Academy of Sciences of the United States of America. Feb, 2002 | Pubmed ID: 11818550 建立区域特异性人多能干细胞衍生星形胶质细胞和神经元模拟ALS的电生理平台 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
建立区域特异性人多能干细胞衍生星形胶质细胞和神经元模拟ALS的电生理平台 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