Nicholas J. Maragakis

Department of Neurology, Johns Hopkins University

Research Area

Biography

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JoVE Journal Publications

ArticleTotal : 1	Year
Publication title Cited by 14	2021

Other Publications

Article	Year
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\| PubMed ID: 11818550	2002
Amyotrophic lateral sclerosis: pathogenesis, differential diagnoses, and potential interventions. The journal of spinal cord medicine\| PubMed ID: 12482167	2002
Topiramate protects against motor neuron degeneration in organotypic spinal cord cultures but not in G93A SOD1 transgenic mice. Neuroscience letters\| PubMed ID: 12566164	2003
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\| PubMed ID: 12832537	2003
Glutamate transporter expression and function in human glial progenitors. Glia\| PubMed ID: 14730707	2004
Altered expression of the glutamate transporter EAAT2b in neurological disease. Annals of neurology\| PubMed ID: 15048885	2004
Glutamate transporters: animal models to neurologic disease. Neurobiology of disease\| PubMed ID: 15056453	2004
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\| PubMed ID: 15329404	2004
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\| PubMed ID: 15519246	2004
Glial restricted precursors protect against chronic glutamate neurotoxicity of motor neurons in vitro. Glia\| PubMed ID: 15657939	2005
Degeneration of respiratory motor neurons in the SOD1 G93A transgenic rat model of ALS. Neurobiology of disease\| PubMed ID: 16084734	2006
Selective up-regulation of the glial Na+-dependent glutamate transporter GLT1 by a neuroimmunophilin ligand results in neuroprotection. Neurobiology of disease\| PubMed ID: 16274998	2006
Loss of the astrocyte glutamate transporter GLT1 modifies disease in SOD1(G93A) mice. Experimental neurology\| PubMed ID: 16753145	2006
Mechanisms of Disease: astrocytes in neurodegenerative disease. Nature clinical practice. Neurology\| PubMed ID: 17117171	2006
Intraparenchymal spinal cord delivery of adeno-associated virus IGF-1 is protective in the SOD1G93A model of ALS. Brain research\| PubMed ID: 17963733	2007
Selective ablation of proliferating astrocytes does not affect disease outcome in either acute or chronic models of motor neuron degeneration. Experimental neurology\| PubMed ID: 18410928	2008
Focal transplantation-based astrocyte replacement is neuroprotective in a model of motor neuron disease. Nature neuroscience\| PubMed ID: 18931666	2008
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\| PubMed ID: 18985465	2009
Rethinking a drug treatment failure on a traditional ALS target. Experimental neurology\| PubMed ID: 19309797	2009
A targeted neuroglial reporter line generated by homologous recombination in human embryonic stem cells. Stem cells (Dayton, Ohio)\| PubMed ID: 19544414	2009
Advances in stem cell research for Amyotrophic Lateral Sclerosis. Current opinion in biotechnology\| PubMed ID: 19819686	2009
Peripheral hyperstimulation alters site of disease onset and course in SOD1 rats. Neurobiology of disease\| PubMed ID: 20381620	2010
Stem cells and the ALS neurologist. Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases\| PubMed ID: 20509745	2010
Spatial and temporal changes in promoter activity of the astrocyte glutamate transporter GLT1 following traumatic spinal cord injury. Journal of neuroscience research\| PubMed ID: 21488085	2011
Reduction in expression of the astrocyte glutamate transporter, GLT1, worsens functional and histological outcomes following traumatic spinal cord injury. Glia\| PubMed ID: 21882244	2011
Stem cell transplantation for spinal cord neurodegeneration. Methods in molecular biology (Clifton, N.J.)\| PubMed ID: 21913120	2011
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\| PubMed ID: 21969586	2011
Human glial-restricted progenitor transplantation into cervical spinal cord of the SOD1 mouse model of ALS. PloS one\| PubMed ID: 21998733	2011
Selective increase of two ABC drug efflux transporters at the blood-spinal cord barrier suggests induced pharmacoresistance in ALS. Neurobiology of disease\| PubMed ID: 22521463	2012
Creation of an open-access, mutation-defined fibroblast resource for neurological disease research. PloS one\| PubMed ID: 22952635	2012
Glia: an emerging target for neurological disease therapy. Stem cell research & therapy\| PubMed ID: 23021042	2012
Exome sequencing to identify de novo mutations in sporadic ALS trios. Nature neuroscience\| PubMed ID: 23708140	2013
Altered astrocytic expression of TDP-43 does not influence motor neuron survival. Experimental neurology\| PubMed ID: 24120466	2013
C9orf72 nucleotide repeat structures initiate molecular cascades of disease. Nature\| PubMed ID: 24598541	2014
Gene profiling of human induced pluripotent stem cell-derived astrocyte progenitors following spinal cord engraftment. Stem cells translational medicine\| PubMed ID: 24604284	2014
Induced pluripotent stem cells from ALS patients for disease modeling. Brain research\| PubMed ID: 25223906	2015
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\| PubMed ID: 25492561	2015
Human glial progenitor engraftment and gene expression is independent of the ALS environment. Experimental neurology\| PubMed ID: 25523812	2015
Peripheral neuropathy in a family with Sandhoff disease and SH3TC2 deficiency. Journal of neurology\| PubMed ID: 25736553	2015
A comprehensive library of familial human amyotrophic lateral sclerosis induced pluripotent stem cells. PloS one\| PubMed ID: 25760436	2015
Neural and glial progenitor transplantation as a neuroprotective strategy for Amyotrophic Lateral Sclerosis (ALS). Brain research\| PubMed ID: 26187754	2015
Human iPS cell-derived astrocyte transplants preserve respiratory function after spinal cord injury. Experimental neurology\| PubMed ID: 26216662	2015
Generation of GFAP::GFP astrocyte reporter lines from human adult fibroblast-derived iPS cells using zinc-finger nuclease technology. Glia\| PubMed ID: 26295203	2016
Hemiparetic Primary Lateral Sclerosis: Revisiting Mills Syndrome. Case reports in neurology\| PubMed ID: 26500542	2015
Primary Lateral Sclerosis and Early Upper Motor Neuron Disease: Characteristics of a Cross-Sectional Population. Journal of clinical neuromuscular disease\| PubMed ID: 26905909	2016
Connexin 43 in astrocytes contributes to motor neuron toxicity in amyotrophic lateral sclerosis. Glia\| PubMed ID: 27083773	2016
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 amyotroph... Glia\| PubMed ID: 27158936	2016
Defining SOD1 ALS natural history to guide therapeutic clinical trial design. Journal of neurology, neurosurgery, and psychiatry\| PubMed ID: 27261500	2017
Serial in vivo imaging of transplanted allogeneic neural stem cell survival in a mouse model of amyotrophic lateral sclerosis. Experimental neurology\| PubMed ID: 28038988	2017
Sporadic ALS Astrocytes Induce Neuronal Degeneration In Vivo. Stem cell reports\| PubMed ID: 28366455	2017
MicroRNA Profiling Reveals Marker of Motor Neuron Disease in ALS Models. The Journal of neuroscience : the official journal of the Society for Neuroscience\| PubMed ID: 28416596	2017
Motoneuron Disease: Basic Science. Advances in neurobiology\| PubMed ID: 28674981	2017
Motoneuron Disease: Clinical. Advances in neurobiology\| PubMed ID: 28674982	2017
What can we learn from the edaravone development program for ALS? Amyotrophic lateral sclerosis & frontotemporal degeneration\| PubMed ID: 28872911	2017
Erratum. Advances in neurobiology\| PubMed ID: 28905310	2017
Fibroblast bioenergetics to classify amyotrophic lateral sclerosis patients. Molecular neurodegeneration\| PubMed ID: 29065921	2017
A randomized controlled trial of resistance and endurance exercise in amyotrophic lateral sclerosis. Amyotrophic lateral sclerosis & frontotemporal degeneration\| PubMed ID: 29191052	2018
Genome-wide Analyses Identify KIF5A as a Novel ALS Gene. Neuron\| PubMed ID: 29566793	2018
A stocked toolbox for understanding the role of astrocytes in disease. Nature reviews. Neurology\| PubMed ID: 29769699	2018
Motor neuron-derived microRNAs cause astrocyte dysfunction in amyotrophic lateral sclerosis. Brain : a journal of neurology\| PubMed ID: 30007309	2018
Olfactory dysfunction in amyotrophic lateral sclerosis. Annals of clinical and translational neurology\| PubMed ID: 30128322	2018
Current and emerging ALS biomarkers: utility and potential in clinical trials. Expert review of neurotherapeutics\| PubMed ID: 30273061	2018
Perfluorocarbon Labeling of Human Glial-Restricted Progenitors for F Magnetic Resonance Imaging. Stem cells translational medicine\| PubMed ID: 30618148	2019
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\| PubMed ID: 31631575	2019
Focal and dose-dependent neuroprotection in ALS mice following AAV2-neurturin delivery. Experimental neurology\| PubMed ID: 31678350	2020
Addressing heterogeneity in amyotrophic lateral sclerosis CLINICAL TRIALS. Muscle & nerve\| PubMed ID: 31899540	2020
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\| PubMed ID: 31988061	2020
Expression and Cellular Distribution of P-Glycoprotein and Breast Cancer Resistance Protein in Amyotrophic Lateral Sclerosis Patients. Journal of neuropathology and experimental neurology\| PubMed ID: 31999342	2020
Amyotrophic lateral sclerosis care and research in the United States during the COVID-19 pandemic: Challenges and opportunities. Muscle & nerve\| PubMed ID: 32445195	2020
Phase 1-2 Trial of Antisense Oligonucleotide Tofersen for ALS. The New England journal of medicine\| PubMed ID: 32640130	2020
Effect of Ezogabine on Cortical and Spinal Motor Neuron Excitability in Amyotrophic Lateral Sclerosis: A Randomized Clinical Trial. JAMA neurology\| PubMed ID: 33226425	2021
Mini-Review: Induced pluripotent stem cells and the search for new cell-specific ALS therapeutic targets. Neuroscience letters\| PubMed ID: 33892003	2021