Jeffrey D Rothstein

Summary

Affiliation: Johns Hopkins University
Country: USA

Publications

  1. ncbi request reprint Beta-lactam antibiotics offer neuroprotection by increasing glutamate transporter expression
    Jeffrey D Rothstein
    Department of Neurology, Johns Hopkins University, Baltimore, Maryland 21287, USA
    Nature 433:73-7. 2005
  2. pmc Analysis of IFT74 as a candidate gene for chromosome 9p-linked ALS-FTD
    Parastoo Momeni
    Laboratory of Neurogenetics, National Institute of Aging, NIH, Bethesda, MD, USA
    BMC Neurol 6:44. 2006
  3. 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
  4. pmc Epigenetic regulation of neuron-dependent induction of astroglial synaptic protein GLT1
    Yongjie Yang
    Department of Neurology, Johns Hopkins University, 600 N Wolfe Street, Baltimore, MD 21287, USA
    Glia 58:277-86. 2010
  5. 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
  6. pmc Presynaptic regulation of astroglial excitatory neurotransmitter transporter GLT1
    Yongjie Yang
    Department of Neurology and Neuroscience, Johns Hopkins University, Baltimore, MD 21287, USA
    Neuron 61:880-94. 2009
  7. 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
  8. doi request reprint The endoplasmic reticulum exit of glutamate transporter is regulated by the inducible mammalian Yip6b/GTRAP3-18 protein
    ALICIA M RUGGIERO
    Department of Neuroscience, Johns Hopkins University, 625 N Wolfe Street, Baltimore, MD 21287, USA
    J Biol Chem 283:6175-83. 2008
  9. pmc Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis
    Shin H Kang
    The Solomon H Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    Nat Neurosci 16:571-9. 2013
  10. pmc Tumor necrosis factor-alpha modulates glutamate transport in the CNS and is a critical determinant of outcome from viral encephalomyelitis
    Jessica Carmen
    Department of Molecular Microbiology and Immunology, Johns Hopkins University Bloomberg School of Public Health, Baltimore, MD 21205, USA
    Brain Res 1263:143-54. 2009

Detail Information

Publications73

  1. ncbi request reprint Beta-lactam antibiotics offer neuroprotection by increasing glutamate transporter expression
    Jeffrey D Rothstein
    Department of Neurology, Johns Hopkins University, Baltimore, Maryland 21287, USA
    Nature 433:73-7. 2005
    ..Thus these studies provide a class of potential neurotherapeutics that act to modulate the expression of glutamate neurotransmitter transporters via gene activation...
  2. pmc Analysis of IFT74 as a candidate gene for chromosome 9p-linked ALS-FTD
    Parastoo Momeni
    Laboratory of Neurogenetics, National Institute of Aging, NIH, Bethesda, MD, USA
    BMC Neurol 6:44. 2006
    ..A new locus for amyotrophic lateral sclerosis--frontotemporal dementia (ALS-FTD) has recently been ascribed to chromosome 9p...
  3. 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
    ....
  4. pmc Epigenetic regulation of neuron-dependent induction of astroglial synaptic protein GLT1
    Yongjie Yang
    Department of Neurology, Johns Hopkins University, 600 N Wolfe Street, Baltimore, MD 21287, USA
    Glia 58:277-86. 2010
    ..In summary, hypermethylation on selective CpG sites of the GLT1 promoter is involved in repression of GLT1 promoter activation, but this regulation does not play a role in astroglial dysfunction of EAAT2 expression in patients with ALS...
  5. 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...
  6. pmc Presynaptic regulation of astroglial excitatory neurotransmitter transporter GLT1
    Yongjie Yang
    Department of Neurology and Neuroscience, Johns Hopkins University, Baltimore, MD 21287, USA
    Neuron 61:880-94. 2009
    ..Presynaptic elements dynamically coordinate normal astroglial function and also provide a fundamental signaling mechanism by which altered neuronal function and injury leads to dysregulated astroglia in CNS disease...
  7. 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...
  8. doi request reprint The endoplasmic reticulum exit of glutamate transporter is regulated by the inducible mammalian Yip6b/GTRAP3-18 protein
    ALICIA M RUGGIERO
    Department of Neuroscience, Johns Hopkins University, 625 N Wolfe Street, Baltimore, MD 21287, USA
    J Biol Chem 283:6175-83. 2008
    ..The features of GTRAP3-18 activity are consistent with recent phylogenic sequence analyses suggesting GTRAP3-18 and JM4 be reclassified as mammalian isoforms of the yeast protein family Yip, Yip6b, and Yip6a, respectively...
  9. pmc Degeneration and impaired regeneration of gray matter oligodendrocytes in amyotrophic lateral sclerosis
    Shin H Kang
    The Solomon H Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    Nat Neurosci 16:571-9. 2013
    ....
  10. pmc Tumor necrosis factor-alpha modulates glutamate transport in the CNS and is a critical determinant of outcome from viral encephalomyelitis
    Jessica Carmen
    Department of Molecular Microbiology and Immunology, Johns Hopkins University Bloomberg School of Public Health, Baltimore, MD 21205, USA
    Brain Res 1263:143-54. 2009
    ..These findings have implications for disease intervention and therapeutic development for the prevention of CNS damage associated with inflammatory responses...
  11. pmc Oligodendroglia metabolically support axons and contribute to neurodegeneration
    Youngjin Lee
    Department of Neurology, The Johns Hopkins University, Baltimore, Maryland 21205, USA
    Nature 487:443-8. 2012
    ..The role of oligodendroglia in axon function and neuron survival has been elusive; this study defines a new fundamental mechanism by which oligodendroglia support neurons and axons...
  12. pmc Zones of enhanced glutamate release from climbing fibers in the mammalian cerebellum
    Martin Paukert
    The Solomon H Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    J Neurosci 30:7290-9. 2010
    ..The enhanced release of glutamate from CFs in Z(+) zones may alter the threshold for synaptic plasticity and prolong inhibition of cerebellar output neurons in deep cerebellar nuclei...
  13. pmc Molecular comparison of GLT1+ and ALDH1L1+ astrocytes in vivo in astroglial reporter mice
    Yongjie Yang
    Department of Neurology, Johns Hopkins University, Baltimore, Maryland 21205, USA
    Glia 59:200-7. 2011
    ..These astroglial reporter mice provide useful in vivo tools for the molecular analysis of astrocytes in physiological and pathological conditions...
  14. 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...
  15. 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...
  16. 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...
  17. 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...
  18. ncbi request reprint Recovery from paralysis in adult rats using embryonic stem cells
    Deepa M Deshpande
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD 21287 6965, USA
    Ann Neurol 60:32-44. 2006
    ..We explored the potential of embryonic stem cell-derived motor neurons to functionally replace those cells destroyed in paralyzed adult rats...
  19. 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...
  20. 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
    ....
  21. doi request reprint Motor neuron disease occurring in a mutant dynactin mouse model is characterized by defects in vesicular trafficking
    Fiona M Laird
    Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    J Neurosci 28:1997-2005. 2008
    ..This novel mouse model will be instrumental for not only clarifying disease mechanisms in ALS, but also for testing therapeutic strategies to ameliorate this devastating disease...
  22. ncbi request reprint Astrocyte glutamate transporters regulate metabotropic glutamate receptor-mediated excitation of hippocampal interneurons
    Yanhua H Huang
    Department of Neuroscience, Johns Hopkins University, Baltimore, Maryland 21205, USA
    J Neurosci 24:4551-9. 2004
    ....
  23. ncbi request reprint Analysis of cerebellar Purkinje cells using EAAT4 glutamate transporter promoter reporter in mice generated via bacterial artificial chromosome-mediated transgenesis
    Dan Gincel
    Department of Neurology, Johns Hopkins University, Baltimore, MD 21287, USA
    Exp Neurol 203:205-12. 2007
    ..The correlation of promoter activity to protein expression makes the EAAT4 BAC promoter reporter a valuable tool to study regulation of EAAT4 expression...
  24. ncbi request reprint Variations in promoter activity reveal a differential expression and physiology of glutamate transporters by glia in the developing and mature CNS
    Melissa R Regan
    Department of Neurology, Johns Hopkins University, Baltimore, Maryland 21287, USA
    J Neurosci 27:6607-19. 2007
    ....
  25. doi request reprint Current hypotheses for the underlying biology of amyotrophic lateral sclerosis
    Jeffrey D Rothstein
    Department of Neurology and Neuroscience, Brain Science Institute, Johns Hopkins University, Baltimore, MD 21287, USA
    Ann Neurol 65:S3-9. 2009
    ..Convergence of these pathways is likely to mediate disease onset and progression...
  26. pmc β-III spectrin is critical for development of purkinje cell dendritic tree and spine morphogenesis
    Yuanzheng Gao
    Department of Neurology and Neuroscience, Johns Hopkins University, Baltimore, Maryland 21205, USA
    J Neurosci 31:16581-90. 2011
    ....
  27. ncbi request reprint Glutamate receptor antagonists protect from virus-induced neural degeneration
    Jennifer L Nargi-Aizenman
    W Harry Feinstone Department of Molecular Microbiology and Immunology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD 21205, USA
    Ann Neurol 55:541-9. 2004
    ..Furthermore, AMPA receptor-mediated motor neuron death is an important contributor to paralysis and mortality in acute alphavirus-induced encephalomyelitis...
  28. 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
    ....
  29. ncbi request reprint The glutamate-aspartate transporter GLAST mediates glutamate uptake at inner hair cell afferent synapses in the mammalian cochlea
    Elisabeth Glowatzki
    Department of Otolaryngology Head and Neck Surgery, Johns Hopkins University, Baltimore, Maryland 21205, USA
    J Neurosci 26:7659-64. 2006
    ..These data indicate that GLAST in supporting cells is responsible for transmitter uptake at IHC afferent synapses...
  30. pmc Reticulon RTN2B regulates trafficking and function of neuronal glutamate transporter EAAC1
    Yiting Liu
    Departments of Neurology and Neuroscience, School of Medicine, Johns Hopkins University, Baltimore, MD 21287, USA
    J Biol Chem 283:6561-71. 2008
    ..These studies indicate that transporter exit from the ER controlled by the interaction with its ER binding partner represents a critical regulatory step in glutamate transporter trafficking to the cell surface...
  31. doi request reprint RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention
    Christopher J Donnelly
    Department of Neurology, Johns Hopkins University, 855 N Wolfe Street, Rangos 2 270, Baltimore, MD 21205, USA Brain Science Institute, Johns Hopkins University, 855 N Wolfe Street, Rangos 2 270, Baltimore, MD 21205, USA
    Neuron 80:415-28. 2013
    ..These data indicate a toxic RNA gain-of-function mechanism as a cause of C9ORF72 ALS and provide candidate antisense therapeutics and candidate human pharmacodynamic markers for therapy. ..
  32. pmc Harmine, a natural beta-carboline alkaloid, upregulates astroglial glutamate transporter expression
    Yun Li
    Department of Neurology, Johns Hopkins University, School of Medicine, Baltimore, MD 21205, USA
    Neuropharmacology 60:1168-75. 2011
    ..Our studies provide potential novel neurotherapeutics by modulating the activity of glutamate transporters via gene activation. This article is part of a Special Issue entitled 'Trends in neuropharmacology: in memory of Erminio Costa'...
  33. pmc Axonal growth of embryonic stem cell-derived motoneurons in vitro and in motoneuron-injured adult rats
    James M Harper
    Department of Neurology, Johns Hopkins University School of Medicine, Pathology 627C, 600 North Wolfe Street, Baltimore, MD 21287, USA
    Proc Natl Acad Sci U S A 101:7123-8. 2004
    ....
  34. pmc Human nasal olfactory epithelium as a dynamic marker for CNS therapy development
    Rita Sattler
    Department of Neurology, Johns Hopkins University, Baltimore, MD 21205, USA
    Exp Neurol 232:203-11. 2011
    ..Therefore, this biomarker approach may provide a reliable evaluation of CNS glial-directed therapies and hopefully improve throughput for nervous system drug discovery...
  35. doi request reprint Models of amyotrophic lateral sclerosis
    Mandy Jackson
    Johns Hopkins University, Baltimore, Maryland, USA
    Curr Protoc Neurosci . 2002
    ..Appropriate animal and cell culture models of ALS can be used to help unravel the sequence of events in motor neuronal degeneration and test potential therapies...
  36. 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...
  37. 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...
  38. pmc NG2+ CNS glial progenitors remain committed to the oligodendrocyte lineage in postnatal life and following neurodegeneration
    Shin H Kang
    The Solomon H Snyder Department of Neuroscience, Johns Hopkins University, Baltimore, MD 21205, USA
    Neuron 68:668-81. 2010
    ..These results indicate that NG2+ cells in the normal CNS are oligodendrocyte precursors with restricted lineage potential and that cell loss and gliosis are not sufficient to alter the lineage potential of these progenitors...
  39. ncbi request reprint Targeting an old mechanism in a new disease-protection of glutamatergic dysfunction in depression
    Rita Sattler
    Department of Neurology, Johns Hopkins University, Baltimore, MD 21287, USA
    Biol Psychiatry 61:137-8. 2007
  40. ncbi request reprint Therapeutic immunization with a glatiramer acetate derivative does not alter survival in G93A and G37R SOD1 mouse models of familial ALS
    Christine Haenggeli
    Department of Neurology, Johns Hopkins University, Baltimore, MD 21287, USA
    Neurobiol Dis 26:146-52. 2007
    ..The biological response in animals, and ultimate clinical translation, will ultimately be dependent on careful and appropriate dose, route and carrier paradigms...
  41. ncbi request reprint Oxidative stress and dopamine deficiency in a genetic mouse model of Lesch-Nyhan disease
    Jasper E Visser
    Department of Neurology, Meyer 6 181, Johns Hopkins Hospital, Baltimore, MD 21287, USA
    Brain Res Dev Brain Res 133:127-39. 2002
    ..Overall, these data provide evidence for increased oxidative stress, but the failure to protect the knockout mice by over-expressing SOD1 argues that oxidative stress is not the sole process responsible for the loss of striatal dopamine...
  42. pmc Localization of a toxic form of superoxide dismutase 1 protein to pathologically affected tissues in familial ALS
    Terrell E Brotherton
    Department of Neurology, Emory University, Atlanta, GA 30322, USA
    Proc Natl Acad Sci U S A 109:5505-10. 2012
    ..The ability of C4F6 to differentiate pathologically affected tissue in mutant SOD1 ALS rodent models and humans, specifically motor neuron populations, suggests that this antibody may recognize a "toxic" form of the mutant SOD1 protein...
  43. 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...
  44. doi request reprint A phase II trial of talampanel in subjects with amyotrophic lateral sclerosis
    Robert M Pascuzzi
    Department of Neurology, Indiana University School of Medicine, Indianapolis, Indiana, USA
    Amyotroph Lateral Scler 11:266-71. 2010
    ..These findings provide strong support for a phase III trial to determine the efficacy of talampanel in subjects with ALS...
  45. ncbi request reprint NAALADase inhibition protects motor neurons against chronic glutamate toxicity
    Ajit G Thomas
    Guilford Pharmaceuticals Inc, 6611 Tributary Street, Baltimore, MD 21224, USA
    Eur J Pharmacol 471:177-84. 2003
    ..Results suggest that NAALADase inhibitors protect against chronic glutamate-mediated motor neuron degeneration and may prove therapeutic towards amyotrophic lateral sclerosis...
  46. ncbi request reprint Climbing fiber activation of EAAT4 transporters and kainate receptors in cerebellar Purkinje cells
    Yanhua H Huang
    Department of Neuroscience, Johns Hopkins University, Baltimore, Maryland 21205, USA
    J Neurosci 24:103-11. 2004
    ..In GluR5 knock-out mice, this current was dramatically reduced, indicating that CF excitation of PCs involves two distinct classes of ionotropic glutamate receptors, AMPA receptors and GluR5-containing kainate receptors...
  47. ncbi request reprint Cyclooxygenase 2 inhibition protects motor neurons and prolongs survival in a transgenic mouse model of ALS
    Daniel B Drachman
    Departments of Neurology and Neuroscience, Johns Hopkins University School of Medicine, Meyer 6 109, 600 N Wolfe Street, Baltimore, MD 21287 7519, USA
    Ann Neurol 52:771-8. 2002
    ..Spinal cords of treated ALS mice showed significant preservation of spinal neurons and diminished astrogliosis and microglial activation. Our results suggest that cyclooxygenase-2 inhibition may benefit ALS patients...
  48. ncbi request reprint Preclinical studies: how much can we rely on?
    Jeffrey D Rothstein
    Johns Hopkins University, USA
    Amyotroph Lateral Scler Other Motor Neuron Disord 5:22-5. 2004
  49. 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...
  50. pmc A neuronal glutamate transporter contributes to neurotransmitter GABA synthesis and epilepsy
    Jehuda P Sepkuty
    Department of Neurology, Johns Hopkins University, Baltimore, Maryland 21287 7247, USA
    J Neurosci 22:6372-9. 2002
    ..EAAC1 may participate in normal GABA neurosynthesis and limbic hyperexcitability, whereas epilepsy can result from a disruption of the interaction between EAAC1 and GABA metabolism...
  51. pmc Oligodendroglia: metabolic supporters of axons
    Brett M Morrison
    Brain Science Institute, Department of Neurology, Johns Hopkins University, Baltimore, MD 21205, USA
    Trends Cell Biol 23:644-51. 2013
    ..New insights into the role of oligodendroglial biology provide an exciting opportunity for revisions in nervous system biology, understanding myelin-based disorders, and therapeutics development. ..
  52. ncbi request reprint Evidence that accumulation of ceramides and cholesterol esters mediates oxidative stress-induced death of motor neurons in amyotrophic lateral sclerosis
    Roy G Cutler
    Laboratory of Neurosciences, National Institute on Aging Gerontology Research Center, Baltimore, MD, USA
    Ann Neurol 52:448-57. 2002
    ..These findings suggest a pivotal role for altered sphingolipid metabolism in the pathogenesis of ALS...
  53. 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...
  54. ncbi request reprint Aquaporin 4 is increased in association with human immunodeficiency virus dementia: implications for disease pathogenesis
    Coryse St Hillaire
    Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    J Neurovirol 11:535-43. 2005
    ..Additional studies may be warranted to determine whether altered AQP4 expression represents a protective and/or maladaptive response to central nervous system (CNS) inflammation...
  55. ncbi request reprint Aggregate formation in Cu,Zn superoxide dismutase-related proteins
    Marjatta Son
    Department of Neurology, University of Texas, Southwestern Medical Center, Dallas 75390, USA
    J Biol Chem 278:14331-6. 2003
    ..CCS- and SOD3-positive inclusions appear to be ubiquitinated and localized to aggresomes. These results suggest that proteins sharing structural similarities to mutant SOD1 are also at risk for aggregate formation...
  56. 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...
  57. ncbi request reprint Paving new pathways
    Jeffrey D Rothstein
    Nat Med 8:938-40. 2002
  58. pmc Amyotrophic lateral sclerosis 2-deficiency leads to neuronal degeneration in amyotrophic lateral sclerosis through altered AMPA receptor trafficking
    Chen Lai
    Laboratory of Neurogenetics, National Institute on Aging, National Institutes of Health, Bethesda, Maryland 20892 3707, USA
    J Neurosci 26:11798-806. 2006
    ....
  59. pmc Translocation of glutamate transporter subtype excitatory amino acid carrier 1 protein in kainic acid-induced rat epilepsy
    Akiko Furuta
    Department of Neuropathology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Am J Pathol 163:779-87. 2003
    ..Our results suggest that the transient EAAC1 internalization participates in the modulation of the transporter function preventing excessive glutamate uptake to pyramidal neurons during the early stage of epilepsy...
  60. ncbi request reprint Reduced expression of glutamate transporter EAAT2 and impaired glutamate transport in human primary astrocytes exposed to HIV-1 or gp120
    Zhuying Wang
    Molecular Virology Division, St Luke s Roosevelt Hospital Center, College of Physicians and Surgeons, Columbia University, New York, NY 10019, USA
    Virology 312:60-73. 2003
    ..Reduction of the ability of HIV-1-infected astrocytes to take up glutamate may contribute to the development of neurological disease...
  61. ncbi request reprint Reality and immortality--neural stem cells for therapies
    Jeffrey D Rothstein
    Nat Biotechnol 22:283-5. 2004
  62. ncbi request reprint Retrograde viral delivery of IGF-1 prolongs survival in a mouse ALS model
    Brian K Kaspar
    Laboratory of Genetics, The Salk Institute for Biological Studies, La Jolla, CA 92037, USA
    Science 301:839-42. 2003
    ..We report that insulin-like growth factor 1 prolongs life and delays disease progression, even when delivered at the time of overt disease symptoms...
  63. ncbi request reprint Regulation of astrocytic glutamate transporter expression by Akt: evidence for a selective transcriptional effect on the GLT-1/EAAT2 subtype
    Li Bin Li
    Department of Pediatrics, University of Pennsylvania, Philadelphia, Pensylvania 19104 4318, USA
    J Neurochem 97:759-71. 2006
    ..From these studies, we conclude that Akt induces the expression of GLT-1 through increased transcription and that Akt can regulate GLT-1 expression without increasing GLAST expression in astrocytes...
  64. ncbi request reprint Expression of glutamate transporter subtypes during normal human corticogenesis and type II lissencephaly
    Akiko Furuta
    Department of Degenerative Neurological Diseases, National Institute of Neuroscience, National Center of Neurology and Psychiatry, 4 1 1, Ogawahigashi, Kodaira, Tokyo 187 8502, Japan
    Brain Res Dev Brain Res 155:155-64. 2005
    ..Altered glutamate transporter expression in type II lissencephaly suggests that glutamate metabolism is involved in the formation of the normal cortex and contributes to the disorganized cortex seen in migration disorders...
  65. ncbi request reprint Spectrin mutations cause spinocerebellar ataxia type 5
    Yoshio Ikeda
    Department of Genetics, Cell Biology, and Development, University of Minnesota, 321 Church St SE, Minneapolis, Minnesota 55455 USA
    Nat Genet 38:184-90. 2006
    ..Spectrin mutations are a previously unknown cause of ataxia and neurodegenerative disease that affect membrane proteins involved in glutamate signaling...
  66. ncbi request reprint Thrombin and lysophosphatidic acid receptors utilize distinct rhoGEFs in prostate cancer cells
    Qin Wang
    Department of Pharmacology, University of Michigan, Ann Arbor, Michigan 48109, USA
    J Biol Chem 279:28831-4. 2004
    ..Suppression of p115rhoGEF had no effect. Thus different rhoGEFs (LARG and PDZrhoGEF) mediate downstream rho signaling by the thrombin and LPA receptors...
  67. ncbi request reprint Retinal colocalization and in vitro interaction of the glutamate transporter EAAT3 and the serum- and glucocorticoid-inducible kinase SGK1 [correction]
    Roman Schniepp
    Department of Physiology I, University of Tubingen, Germany
    Invest Ophthalmol Vis Sci 45:1442-9. 2004
    ..EAAT3 is expressed in retinal ganglion cells and accomplishes the clearance of glutamate from synaptic clefts...
  68. ncbi request reprint Mechanisms for reversible regulation between G13 and Rho exchange factors
    Clark D Wells
    Department of Pharmacology, University of Texas, Southwestern Medical Center at Dallas, Texas 75390 9041, USA
    J Biol Chem 277:1174-81. 2002
    ..These results suggest a mechanism for activation of the nucleotide exchange activity of p115 RhoGEF that involves direct and coordinate interaction of Galpha(13) to both its RGS and DH domains...
  69. ncbi request reprint Of mice and men: reconciling preclinical ALS mouse studies and human clinical trials
    Jeffrey D Rothstein
    Ann Neurol 53:423-6. 2003
  70. ncbi request reprint Glucocorticoid regulation of GLT-1 glutamate transporter isoform expression in the rat hippocampus
    Anita E Autry
    Department of Pharmacology, Physiology and Neuroscience, School of Medicine, University of South Carolina, Columbia, SC 29208, USA
    Neuroendocrinology 83:371-9. 2006
    ..These studies suggest that GLT-1b expression is regulated by stress levels of glucocorticoids (GCs) and GLT-1 expression is regulated by stress-induced increases in extracellular glutamate levels in the CA3 region...
  71. ncbi request reprint Interaction of PDZRhoGEF with microtubule-associated protein 1 light chains: link between microtubules, actin cytoskeleton, and neuronal polarity
    David M Longhurst
    Centre for Neuroscience Research, The University of Edinburgh, Edinburgh EH9 1QH, Scotland, United Kingdom
    J Biol Chem 281:12030-40. 2006
    ..This coordinated action of the actin and microtubular cytoskeletons is essential for the development and maintenance of neuronal polarity...
  72. ncbi request reprint TDP-43 in amyotrophic lateral sclerosis: pathophysiology or patho-babel?
    Jeffrey D Rothstein
    Ann Neurol 61:382-4. 2007
  73. ncbi request reprint Signal-crosstalk between Rho/ROCK and c-Jun NH2-terminal kinase mediates migration of vascular smooth muscle cells stimulated by angiotensin II
    Haruhiko Ohtsu
    Cardiovascular Research Center, Temple University School of Medicine, 3420 N Broad St, Philadelphia, PA 19140, USA
    Arterioscler Thromb Vasc Biol 25:1831-6. 2005
    ..Here, we tested the hypothesis that Rho/ROCK are critically involved in angiotensin II (Ang II)-induced migration of vascular smooth muscle cells (VSMCs) by mediating a specific signal cross-talk...