Nicholas J Maragakis

Summary

Affiliation: Johns Hopkins University
Country: USA

Publications

  1. ncbi request reprint Human embryonic germ cell derivatives facilitate motor recovery of rats with diffuse motor neuron injury
    Douglas A Kerr
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
    J Neurosci 23:5131-40. 2003
  2. ncbi request reprint Glutamate transporters: animal models to neurologic disease
    Nicholas J Maragakis
    Department of Neurology, Johns Hopkins University, Baltimore, MD 21287, USA
    Neurobiol Dis 15:461-73. 2004
  3. doi request reprint Stem cells and the ALS neurologist
    Nicholas J Maragakis
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Amyotroph Lateral Scler 11:417-23. 2010
  4. pmc Focal transplantation-based astrocyte replacement is neuroprotective in a model of motor neuron disease
    Angelo C Lepore
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
    Nat Neurosci 11:1294-301. 2008
  5. pmc Human glial-restricted progenitor transplantation into cervical spinal cord of the SOD1 mouse model of ALS
    Angelo C Lepore
    Department of Neuroscience, Thomas Jefferson University Medical College, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 6:e25968. 2011
  6. doi request reprint Rethinking a drug treatment failure on a traditional ALS target
    Nicholas J Maragakis
    Department of Neurology, Johns Hopkins University, School of Medicine, 600 N Wolfe St, Meyer 6 119, Baltimore, MD 21287, USA
    Exp Neurol 216:254-7. 2009
  7. ncbi request reprint Mechanisms of Disease: astrocytes in neurodegenerative disease
    Nicholas J Maragakis
    Department of Neurology, Johns Hopkins University School of Medicine, 600 N Wolfe Street, Meyer 6 119, Baltimore, MD 21287, USA
    Nat Clin Pract Neurol 2:679-89. 2006
  8. ncbi request reprint Glial restricted precursors protect against chronic glutamate neurotoxicity of motor neurons in vitro
    Nicholas J Maragakis
    Department of Neurology and Neuroscience, Johns Hopkins University, Baltimore, Maryland 21287, USA
    Glia 50:145-59. 2005
  9. ncbi request reprint Degeneration of respiratory motor neurons in the SOD1 G93A transgenic rat model of ALS
    Jeronia Llado
    Department of Neurology, Johns Hopkins University 600 N Wolfe Street, Meyer 6 119, Baltimore, MD 21287, USA
    Neurobiol Dis 21:110-8. 2006
  10. ncbi request reprint Viral-induced spinal motor neuron death is non-cell-autonomous and involves glutamate excitotoxicity
    Jessica Darman
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
    J Neurosci 24:7566-75. 2004

Research Grants

Collaborators

Detail Information

Publications38

  1. ncbi request reprint Human embryonic germ cell derivatives facilitate motor recovery of rats with diffuse motor neuron injury
    Douglas A Kerr
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
    J Neurosci 23:5131-40. 2003
    ..We conclude that cells derived from human pluripotent stem cells have the capacity to restore neurologic function in animals with diffuse motor neuron disease via enhancement of host neuron survival and function...
  2. ncbi request reprint Glutamate transporters: animal models to neurologic disease
    Nicholas J Maragakis
    Department of Neurology, Johns Hopkins University, Baltimore, MD 21287, USA
    Neurobiol Dis 15:461-73. 2004
    ..Recent work has focused on glutamate transporter biology in human diseases with an emphasis on how manipulation of these transporter proteins may lead to therapeutic interventions in neurologic disease...
  3. doi request reprint Stem cells and the ALS neurologist
    Nicholas J Maragakis
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Amyotroph Lateral Scler 11:417-23. 2010
    ..Although the therapeutic role of stems cells in ALS and other neurodegenerations remains to be established, recent experimental data provide grounds for considered optimism...
  4. pmc Focal transplantation-based astrocyte replacement is neuroprotective in a model of motor neuron disease
    Angelo C Lepore
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
    Nat Neurosci 11:1294-301. 2008
    ....
  5. pmc Human glial-restricted progenitor transplantation into cervical spinal cord of the SOD1 mouse model of ALS
    Angelo C Lepore
    Department of Neuroscience, Thomas Jefferson University Medical College, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 6:e25968. 2011
    ....
  6. doi request reprint Rethinking a drug treatment failure on a traditional ALS target
    Nicholas J Maragakis
    Department of Neurology, Johns Hopkins University, School of Medicine, 600 N Wolfe St, Meyer 6 119, Baltimore, MD 21287, USA
    Exp Neurol 216:254-7. 2009
    ....
  7. ncbi request reprint Mechanisms of Disease: astrocytes in neurodegenerative disease
    Nicholas J Maragakis
    Department of Neurology, Johns Hopkins University School of Medicine, 600 N Wolfe Street, Meyer 6 119, Baltimore, MD 21287, USA
    Nat Clin Pract Neurol 2:679-89. 2006
    ..These models have led to the development of targeted therapies for pathways in which astrocytes participate, and this research should ultimately influence the clinical treatment of neurodegenerative disorders...
  8. ncbi request reprint Glial restricted precursors protect against chronic glutamate neurotoxicity of motor neurons in vitro
    Nicholas J Maragakis
    Department of Neurology and Neuroscience, Johns Hopkins University, Baltimore, Maryland 21287, USA
    Glia 50:145-59. 2005
    ..The seeding of G3s resulted in a reduction of motor neuron cell death. Hence, we believe that these cells may potentially play a role in cell-based neuroprotection from glutamate excitotoxicity...
  9. ncbi request reprint Degeneration of respiratory motor neurons in the SOD1 G93A transgenic rat model of ALS
    Jeronia Llado
    Department of Neurology, Johns Hopkins University 600 N Wolfe Street, Meyer 6 119, Baltimore, MD 21287, USA
    Neurobiol Dis 21:110-8. 2006
    ..Taken together, these data suggest that respiratory motor neuron loss results in significant electrophysiologic changes and diaphragmatic atrophy. These changes may play a significant role resulting in death of these animals...
  10. ncbi request reprint Viral-induced spinal motor neuron death is non-cell-autonomous and involves glutamate excitotoxicity
    Jessica Darman
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
    J Neurosci 24:7566-75. 2004
    ..Similar changes may occur in other motor neuron disorders such as amyotrophic lateral sclerosis or West Nile Virus-induced poliomyelitis, suggesting a common tissue injury pathway...
  11. ncbi request reprint Loss of the astrocyte glutamate transporter GLT1 modifies disease in SOD1(G93A) mice
    Andrea C Pardo
    Department of Neurology, Johns Hopkins University, 600 N Wolfe St, Meyer 6 119, Baltimore, MD 21287, USA
    Exp Neurol 201:120-30. 2006
    ..This study suggests that astrocytes, and the astrocyte glutamate transporter GLT1, play a role in modifying disease progression and motor neuron loss in this model...
  12. ncbi request reprint Adult glial precursor proliferation in mutant SOD1G93A mice
    Tim Magnus
    Laboratory of Neurosciences, National Institute on Aging Intramural Research Program, National Institutes of Health, Baltimore, Maryland 21287, USA
    Glia 56:200-8. 2008
    ..These data suggest that adult glial progenitors from SOD1G93A mice differentially respond to inflammatory cytokines and contribute to the observed reactive astrocytosis observed in SOD1G93A mouse lumbar spinal cord...
  13. doi request reprint Spatial and temporal changes in promoter activity of the astrocyte glutamate transporter GLT1 following traumatic spinal cord injury
    Angelo C Lepore
    Department of Neurology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    J Neurosci Res 89:1001-17. 2011
    ..Results also suggest that following SCI a significant portion of astrocytes lacks GLT1 expression, possibly compromising the important role of astrocytes in glutamate homeostasis...
  14. ncbi request reprint Selective up-regulation of the glial Na+-dependent glutamate transporter GLT1 by a neuroimmunophilin ligand results in neuroprotection
    Raquelli Ganel
    Department of Neurology, Johns Hopkins University, Baltimore, MD 21287, USA
    Neurobiol Dis 21:556-67. 2006
    ..These studies suggest that neuroimmunophilins can regulate GLT1 and that their ligands could serve as therapies for neurodegenerative disorders...
  15. ncbi request reprint Impaired SDF1/CXCR4 signaling in glial progenitors derived from SOD1(G93A) mice
    Yongquan Luo
    Laboratory of Neurosciences, National Institute on Aging Intramural Research Program, Baltimore, Maryland, USA
    J Neurosci Res 85:2422-32. 2007
    ..These data indicate that the abnormalities in SOD1(G93A) glial progenitor expression of CXCR4 and its mediated signaling and function occur during spinal cord development and highlight nonneuronal (glial) abnormalities in this ALS model...
  16. pmc Peripheral hyperstimulation alters site of disease onset and course in SOD1 rats
    Angelo C Lepore
    Department of Neurology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
    Neurobiol Dis 39:252-64. 2010
    ....
  17. ncbi request reprint Electrical impedance myography correlates with standard measures of Als severity
    Seward B Rutkove
    Department of Neurology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, TCC 810, Boston, Massachusetts, USA, 02215
    Muscle Nerve 49:441-3. 2014
    ..Conclusions: These results support the concept that EIM can serve as a meaningful measure of disease severity in ALS. Muscle Nerve 49:441-443, 2014. ..
  18. pmc Intraparenchymal spinal cord delivery of adeno-associated virus IGF-1 is protective in the SOD1G93A model of ALS
    Angelo C Lepore
    Department of Neurology, The Johns Hopkins University School of Medicine, 600 N Wolfe St, Meyer 6 109, Baltimore, MD 21287, USA
    Brain Res 1185:256-65. 2007
    ....
  19. doi request reprint Selective ablation of proliferating astrocytes does not affect disease outcome in either acute or chronic models of motor neuron degeneration
    Angelo C Lepore
    Department of Neurology, The Johns Hopkins University School of Medicine, 600 North Wolfe Street, Meyer 6 119, Baltimore, MD 21287, USA
    Exp Neurol 211:423-32. 2008
    ..These findings demonstrate that while normal astrocyte function is an important factor in the etiology of motor neuron diseases such as ALS, astrocyte proliferation itself does not play a significant role...
  20. pmc Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo
    Sophia T Papadeas
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA
    Proc Natl Acad Sci U S A 108:17803-8. 2011
    ..The SOD1(G93A) astrocyte-induced MN death seemed in part mediated by host microglial activation. These findings show that mSOD1 astrocytes alone can induce WT MN death and associated pathological changes in vivo...
  21. ncbi request reprint Neural stem cells protect against glutamate-induced excitotoxicity and promote survival of injured motor neurons through the secretion of neurotrophic factors
    Jeronia Llado
    Department of Neurology and Neuroscience, Johns Hopkins University, Baltimore, MD 21287, USA
    Mol Cell Neurosci 27:322-31. 2004
    ..We also showed that NSCs can protect spinal cord cultures from experimentally induced excitotoxic damage. The neuroprotective potential of NSCs was further confirmed in vivo by their ability to protect against motor neuron cell death...
  22. pmc Reduction in expression of the astrocyte glutamate transporter, GLT1, worsens functional and histological outcomes following traumatic spinal cord injury
    Angelo C Lepore
    Department of Neuroscience, Thomas Jefferson University Medical College, 900 Walnut St, Jefferson Hospital for Neuroscience, Philadelphia, Pennsylvania, USA
    Glia 59:1996-2005. 2011
    ..These findings also suggest that increasing intraspinal GLT1 expression may represent a therapeutically relevant target for SCI treatment...
  23. ncbi request reprint Glutamate transporter expression and function in human glial progenitors
    Nicholas J Maragakis
    Department of Neurology and Neuroscience, Johns Hopkins University, Meyer 6 109, 600 N Wolfe Street, Baltimore, MD 21287 5953, USA
    Glia 45:133-43. 2004
    ....
  24. doi request reprint Altered astrocytic expression of TDP-43 does not influence motor neuron survival
    Amanda M Haidet-Phillips
    Department of Neurology, Johns Hopkins University School of Medicine, Rangos 248, 855 North Wolfe Street, Baltimore, MD 21205, USA
    Exp Neurol 250:250-9. 2013
    ..Our study reinforces the heterogeneity in ALS disease mechanisms and highlights the potential for future screening subsets of ALS patients prior to treatment with cell type-directed therapies. ..
  25. pmc Gene profiling of human induced pluripotent stem cell-derived astrocyte progenitors following spinal cord engraftment
    Amanda M Haidet-Phillips
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA Departments of Rehabilitation and Regenerative Medicine, Pathology and Cell Biology, Neurology, and Neuroscience, Center for Motor Neuron Biology and Disease, Columbia Stem Cell Initiative, Columbia University Medical Center, New York, New York, Stem Cell Laboratory for CNS Disease Modeling MultiPark, Department of Experimental Medical Science, Lund University, Lund, Sweden Howard Hughes Medical Institute, Harvard Stem Cell Institute and Department of Stem Cell and Regenerative Biology, Harvard University, Cambridge, Massachusetts, USA
    Stem Cells Transl Med 3:575-85. 2014
    ....
  26. pmc Electrical impedance myography as a biomarker to assess ALS progression
    Seward B Rutkove
    Department of Neurology, Beth Israel Deaconess Medical Center, Boston, Massachusetts 02215, USA
    Amyotroph Lateral Scler 13:439-45. 2012
    ..In conclusion, EIM can serve as a useful ALS biomarker that offers the prospect of greatly accelerating phase 2 clinical trials...
  27. doi request reprint Advances in stem cell research for Amyotrophic Lateral Sclerosis
    Sophia T Papadeas
    Johns Hopkins University, 600 N Wolfe St, Meyer 6 119, Baltimore, MD 21287, USA
    Curr Opin Biotechnol 20:545-51. 2009
    ..The use of iPSCs from ALS patients will allow for potential autologous cell transplantation, drug discovery, and an increased understanding of ALS pathobiology...
  28. doi request reprint Stem cell transplantation for spinal cord neurodegeneration
    Angelo C Lepore
    Department of Neurology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA
    Methods Mol Biol 793:479-93. 2011
    ..The SOD1G93A rats and mice are currently the most well-studied animal model of the disease...
  29. ncbi request reprint Altered expression of the glutamate transporter EAAT2b in neurological disease
    Nicholas J Maragakis
    Departments of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA
    Ann Neurol 55:469-77. 2004
    ..These studies clearly document altered regulation and splicing of the dominant glutamate transporter EAAT2 under conditions of neurological stress...
  30. pmc C9orf72 nucleotide repeat structures initiate molecular cascades of disease
    Aaron R Haeusler
    1 Department of Biochemistry and Molecular Biology, Johns Hopkins University Baltimore, Maryland 21205, USA 2 Department of Neuroscience, Johns Hopkins University Baltimore, Maryland 21205, USA
    Nature 507:195-200. 2014
    ....
  31. ncbi request reprint Topiramate protects against motor neuron degeneration in organotypic spinal cord cultures but not in G93A SOD1 transgenic mice
    Nicholas J Maragakis
    Johns Hopkins University, Department of Neurology, Meyer 6 109, 600 North Wolfe Street, Baltimore, MD 21287, USA
    Neurosci Lett 338:107-10. 2003
    ..These studies suggest that topiramate could be useful as a neuroprotectant, but were not effective in more complex motor injury paradigms such as the mouse model of ALS...
  32. pmc Selective increase of two ABC drug efflux transporters at the blood-spinal cord barrier suggests induced pharmacoresistance in ALS
    Michael R Jablonski
    Weinberg Unit for ALS Research, Farber Institute for Neuroscience, Department of Neuroscience, Thomas Jefferson University, Philadelphia, PA 19107, USA
    Neurobiol Dis 47:194-200. 2012
    ..Our observations have large implications for ALS therapeutics in humans and suggest that the obstacle provided by these transporters to drug treatments must be overcome to develop effective ALS pharmacotherapies...
  33. pmc Selected statins produce rapid spinal motor neuron loss in vitro
    Beth B Murinson
    Department of Neurology, Johns Hopkins School of Medicine, Baltimore, USA
    BMC Musculoskelet Disord 13:100. 2012
    ..Although some have postulated class-effects, prior studies of hepatocytes and myocytes indicate that the statins may exhibit differential effects. Studies of neuronal cells have been limited...
  34. doi request reprint Accurate ALSFRS-R scores can be generated from retrospective review of clinic notes
    Noah Lechtzin
    Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    Amyotroph Lateral Scler 10:244-7. 2009
    ..This study indicates that ALSFRS-R scores can be accurately reproduced from information in clinic notes and should be considered as a marker of disease severity for use in retrospective studies...
  35. ncbi request reprint Amyotrophic lateral sclerosis: pathogenesis, differential diagnoses, and potential interventions
    Sarju A Patel
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA
    J Spinal Cord Med 25:262-73. 2002
    ..Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative motor neuron disease. The etiology is likely multifactorial, involving both genetic and environmental factors...
  36. ncbi request reprint A novel secretory factor, Neurogenesin-1, provides neurogenic environmental cues for neural stem cells in the adult hippocampus
    Takatoshi Ueki
    Department of Anatomy and Neuroscience, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka 431 3192, Japan
    J Neurosci 23:11732-40. 2003
    ..These findings suggest endogenous mechanisms that induce adult hippocampal neurogenesis and propose an innovative treatment for the neurodegenerative diseases that cause loss of hippocampal neurons...
  37. pmc Amyotrophic lateral sclerosis: an emerging era of collaborative gene discovery
    Katrina Gwinn
    National Institute for Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS ONE 2:e1254. 2007
    ..This resource should facilitate genetic discoveries which we anticipate will ultimately provide a better understanding of the biological mechanisms of neurodegeneration in ALS...
  38. pmc Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS)
    David S Howland
    Department of Molecular Genetics, Wyeth Research, CN8000, Princeton, NJ 08543, USA
    Proc Natl Acad Sci U S A 99:1604-9. 2002
    ..These transgenic rats provide a valuable resource to pursue experimentation and therapeutic development, currently difficult or impossible to perform with existing ALS transgenic mice...

Research Grants1

  1. The Glutamate Transporter EAAT4 in Purkinje Cell Biology
    Nicholas Maragakis; Fiscal Year: 2003
    ..abstract not available ..