neurofilament proteins

Summary

Summary: Type III intermediate filament proteins that assemble into neurofilaments, the major cytoskeletal element in nerve axons and dendrites. They consist of three distinct polypeptides, the neurofilament triplet. Types I, II, and IV intermediate filament proteins form other cytoskeletal elements such as keratins and lamins. It appears that the metabolism of neurofilaments is disturbed in Alzheimer's disease, as indicated by the presence of neurofilament epitopes in the neurofibrillary tangles, as well as by the severe reduction of the expression of the gene for the light neurofilament subunit of the neurofilament triplet in brains of Alzheimer's patients. (Can J Neurol Sci 1990 Aug;17(3):302)

Top Publications

  1. ncbi Neurofilament phosphoforms: surrogate markers for axonal injury, degeneration and loss
    Axel Petzold
    Department of Neuroimmunology, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK
    J Neurol Sci 233:183-98. 2005
  2. ncbi Mitochondrial dysfunction as a cause of axonal degeneration in multiple sclerosis patients
    Ranjan Dutta
    Department of Neurosciences, Lerner Research Institute, Cleveland Clinic Foundation, OH 44195, USA
    Ann Neurol 59:478-89. 2006
  3. ncbi Axonal damage in relapsing multiple sclerosis is markedly reduced by natalizumab
    Martin Gunnarsson
    Department of Neurology, Orebro University Hospital, Orebro, Goteborg
    Ann Neurol 69:83-9. 2011
  4. ncbi Combination of CSF N-acetylaspartate and neurofilaments in multiple sclerosis
    C E Teunissen
    Molecular Cell Biology and Immunology, VU University Medical Center, FdG, P O Box 7057, 1007 MB Amsterdam, The Netherlands
    Neurology 72:1322-9. 2009
  5. ncbi The neurofilament heavy chain (NfH) in the cerebrospinal fluid diagnosis of Alzheimer's disease
    Johannes Brettschneider
    Department of Neurology, University of Ulm, Ulm, Germany
    Dement Geriatr Cogn Disord 21:291-5. 2006
  6. ncbi Neurofilament heavy chain in CSF correlates with relapses and disability in multiple sclerosis
    J Kuhle
    Department of Biomedicine and Neurology, University Hospital Basel, CH 4031 Basel, Switzerland
    Neurology 76:1206-13. 2011
  7. ncbi An essential cytoskeletal linker protein connecting actin microfilaments to intermediate filaments
    Y Yang
    Howard Hughes Medical Institute, Department of Molecular Genetics and Cell Biology, The University of Chicago, Illinois 60637, USA
    Cell 86:655-65. 1996
  8. ncbi Neurofilaments bind tubulin and modulate its polymerization
    Arnaud Bocquet
    Laboratoire Neurobiologie and Transgenese, Unité Propre de Recherche de l Enseignement Supérieur EA3143, INSERM, Centre Hospitalier Universitaire, 49033 Angers, France
    J Neurosci 29:11043-54. 2009
  9. ncbi Activated microglia mediate axoglial disruption that contributes to axonal injury in multiple sclerosis
    Owain W Howell
    Centre for Neuroscience, Division of Experimental Medicine, Imperial College Faculty of Medicine, Hammersmith Hospital Campus, London, UK
    J Neuropathol Exp Neurol 69:1017-33. 2010
  10. ncbi Phagocytosis of neuronal debris by microglia is associated with neuronal damage in multiple sclerosis
    Ruth Huizinga
    Department of Immunology, Erasmus MC, University Medical Centre, Rotterdam, The Netherlands
    Glia 60:422-31. 2012

Research Grants

  1. Dysfunction of neurofilaments in disease: an iPS model of Giant Axonal Neuropathy
    BETHANY LESLIE JOHNSON-KERNER; Fiscal Year: 2012
  2. Pathogenesis Of Essential Tremor: Cerebellar Metabolism
    Elan D Louis; Fiscal Year: 2012
  3. MOLECULAR MARKERS OF PROGNOSIS IN MEDULLOBLASTOMA
    SANDRA BIGNER; Fiscal Year: 2000
  4. CALMODULIN DEPENDENT KINASE AND SYNAPTIC FUNCTION
    MARY VALLANO; Fiscal Year: 1992
  5. MECHANISMS OF OCCUPATIONAL NEUROPATHIES
    MOHAMED ABOU DONIA; Fiscal Year: 1999
  6. FAMILIAL ALS--MECHANISM OF INITIATION
    Dale Bredesen; Fiscal Year: 1999
  7. INTERMEDIATE FILAMENTS--MOLECULAR APPROACH TO FUNCTION
    STEPHEN TAPSCOTT; Fiscal Year: 1990
  8. Calpain Inhibition Strategies for Spinal Cord Injury
    JAMES GEDDES; Fiscal Year: 2007
  9. MECHANISMS OF DYING BACK NEUROPATHIES
    MOHAMED ABOU DONIA; Fiscal Year: 1993
  10. TOXIC MODELS OF NEUROFIBRILLARY PATHOLOGY
    Douglas Anthony; Fiscal Year: 1991

Detail Information

Publications265 found, 100 shown here

  1. ncbi Neurofilament phosphoforms: surrogate markers for axonal injury, degeneration and loss
    Axel Petzold
    Department of Neuroimmunology, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK
    J Neurol Sci 233:183-98. 2005
    ..With reference to the present symposium on multiple sclerosis, the current literature on body fluid levels of neurofilaments in demyelinating disease is summarised...
  2. ncbi Mitochondrial dysfunction as a cause of axonal degeneration in multiple sclerosis patients
    Ranjan Dutta
    Department of Neurosciences, Lerner Research Institute, Cleveland Clinic Foundation, OH 44195, USA
    Ann Neurol 59:478-89. 2006
    ..Development of neuroprotective therapies will require elucidation of the molecular mechanisms by which neurons and axons degenerate...
  3. ncbi Axonal damage in relapsing multiple sclerosis is markedly reduced by natalizumab
    Martin Gunnarsson
    Department of Neurology, Orebro University Hospital, Orebro, Goteborg
    Ann Neurol 69:83-9. 2011
    ..Therefore, we studied the effect of natalizumab treatment on the release of 2 brain-specific tissue damage markers into cerebrospinal fluid (CSF) in MS patients...
  4. ncbi Combination of CSF N-acetylaspartate and neurofilaments in multiple sclerosis
    C E Teunissen
    Molecular Cell Biology and Immunology, VU University Medical Center, FdG, P O Box 7057, 1007 MB Amsterdam, The Netherlands
    Neurology 72:1322-9. 2009
    ..Next, we wanted to know the additional value of measurement of NAA compared to other candidate markers for axonal damage, such as neurofilament subunits and tau protein...
  5. ncbi The neurofilament heavy chain (NfH) in the cerebrospinal fluid diagnosis of Alzheimer's disease
    Johannes Brettschneider
    Department of Neurology, University of Ulm, Ulm, Germany
    Dement Geriatr Cogn Disord 21:291-5. 2006
    ....
  6. ncbi Neurofilament heavy chain in CSF correlates with relapses and disability in multiple sclerosis
    J Kuhle
    Department of Biomedicine and Neurology, University Hospital Basel, CH 4031 Basel, Switzerland
    Neurology 76:1206-13. 2011
    ..We sought to discover whether CSF levels of neurofilament heavy chain protein (NfH(SMI35)) correlate with disability, disease activity, or specific stages of MS...
  7. ncbi An essential cytoskeletal linker protein connecting actin microfilaments to intermediate filaments
    Y Yang
    Howard Hughes Medical Institute, Department of Molecular Genetics and Cell Biology, The University of Chicago, Illinois 60637, USA
    Cell 86:655-65. 1996
    ..In BPAG1 null mice, axonal architecture is markedly perturbed, consistent with a failure to tether neurofilaments to the actin cytoskeleton and underscoring the physiological relevance of this protein...
  8. ncbi Neurofilaments bind tubulin and modulate its polymerization
    Arnaud Bocquet
    Laboratoire Neurobiologie and Transgenese, Unité Propre de Recherche de l Enseignement Supérieur EA3143, INSERM, Centre Hospitalier Universitaire, 49033 Angers, France
    J Neurosci 29:11043-54. 2009
    ..This capacity also suggests novel mechanisms through which inherited or acquired disruptions in intermediate filaments might contribute to pathogenesis in multiple conditions...
  9. ncbi Activated microglia mediate axoglial disruption that contributes to axonal injury in multiple sclerosis
    Owain W Howell
    Centre for Neuroscience, Division of Experimental Medicine, Imperial College Faculty of Medicine, Hammersmith Hospital Campus, London, UK
    J Neuropathol Exp Neurol 69:1017-33. 2010
    ....
  10. ncbi Phagocytosis of neuronal debris by microglia is associated with neuronal damage in multiple sclerosis
    Ruth Huizinga
    Department of Immunology, Erasmus MC, University Medical Centre, Rotterdam, The Netherlands
    Glia 60:422-31. 2012
    ..Although uptake is essential for clearing neuronal debris, phagocytic cells could also play a role in augmenting autoimmunity to neuronal antigens...
  11. ncbi Neurofilament protein in cerebrospinal fluid: a marker of white matter changes
    M Sjogren
    Institute of Clinical Neuroscience, Goteborg University, Goteborg, Sweden
    J Neurosci Res 66:510-6. 2001
    The objective of this study was to compare cerebrospinal fluid (CSF) levels of the light subtype of the neurofilament proteins (NFL), tau, and beta-amyloid42 (Abeta42) in individuals with moderate or severe white matter changes (WMC) and ..
  12. ncbi Development of chromaffin cells depends on MASH1 function
    Katrin Huber
    Neuroanatomy, Interdisciplinary Center for Neurosciences IZN, University of Heidelberg, INF 307, D 69120 Heidelberg, Germany
    Development 129:4729-38. 2002
    ..Together, these findings suggest that the development of chromaffin cells does depend on MASH1 function not only for catecholaminergic differentiation but also for general chromaffin cell differentiation...
  13. ncbi pNfH is a promising biomarker for ALS
    Jeban Ganesalingam
    King s Health Partners Centre for Neurodegeneration Research, Institute of Psychiatry, Kings College London, UK
    Amyotroph Lateral Scler Frontotemporal Degener 14:146-9. 2013
    ..1247, p = 0.001). In conclusion, pNfH represents a promising candidate for inclusion in a panel of diagnostic and prognostic biomarkers...
  14. ncbi T-cell responses to neurofilament light protein are part of the normal immune repertoire
    Ruth Huizinga
    Department of Immunobiology, Biomedical Primate Research Centre, Rijswijk, The Netherlands
    Int Immunol 21:433-41. 2009
    ..The finding of T-cell responses to NF-L in MS patients may reflect transient activation of pathogenic potential but their presence also in healthy controls indicates that these cells are part of the normal immune repertoire...
  15. ncbi The gene encoding gigaxonin, a new member of the cytoskeletal BTB/kelch repeat family, is mutated in giant axonal neuropathy
    P Bomont
    Institut de Genetique et de Biologie Moleculaire et Cellulaire, CNRS INSERM ULP, B P 163, 67404 Illkirch Cedex, C U de Strasbourg, France
    Nat Genet 26:370-4. 2000
    ....
  16. ncbi Increased expression of cdk5/p25 in N2a cells leads to hyperphosphorylation and impaired axonal transport of neurofilament proteins
    Jie Zhou
    Department of Biochemistry and Molecular Biology, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, China
    Life Sci 86:532-7. 2010
    ..The increased cdk5 expression and enhanced phosphorylation of tau and NFs have been seen in AD patients. Our study aimed at investigating the effects of increased cdk5 activity on axonal transport of neurofilaments (NFs)...
  17. ncbi Neurochemical aftermath of amateur boxing
    Henrik Zetterberg
    Department of Experimental Neuroscience, The Sahlgrenska Academy at Goteborg University, Goteborg, Sweden
    Arch Neurol 63:1277-80. 2006
    ..Little solid information is available on the possible risks for neuronal injury in amateur boxing...
  18. ncbi Hyperphosphorylated neurofilament NF-H is a serum biomarker of axonal injury
    Gerry Shaw
    Department of Neuroscience, University of Florida College of Medicine, Gainesville, USA
    Biochem Biophys Res Commun 336:1268-77. 2005
    ..These findings suggest that serum levels of pNF-H immunoreactivity may be used to conveniently monitor neuronal damage and degeneration in experimental and presumably clinical situations...
  19. ncbi Integrators of the cytoskeleton that stabilize microtubules
    Y Yang
    Howard Hughes Medical Institute, Department of Molecular Genetics and Cell Biology, Chicago, Illinois 60637, USA
    Cell 98:229-38. 1999
    ..Its isoforms interconnect all three cytoskeletal networks, a feature apparently central to neuronal survival...
  20. pmc CSF neurofilament proteins in the differential diagnosis of dementia
    D De Jong
    Department of Neurology, Radboud University Nijmegen Medical Centre, 6500 HB, Nijmegen, The Netherlands
    J Neurol Neurosurg Psychiatry 78:936-8. 2007
    ..Neurofilament (NF) proteins are major cytoskeletal constituents of neurons. Increased CSF NF levels may reflect neuronal degeneration...
  21. ncbi Retinoic acid regulates myelin formation in the peripheral nervous system
    Maria Jesus Latasa
    Department of Endocrine and Nervous System Physiopathology, Instituto de Investigaciones Biomedicas, Universidad Autonoma de Madrid, Madrid, Spain
    Glia 58:1451-64. 2010
    ..At the same time, through a RAR-dependent mechanism, RA downregulates myelin-associated glycoprotein, which also contributes to the dysmyelinating effect of the retinoid...
  22. pmc Combination of neurofilament heavy chain and complement C3 as CSF biomarkers for ALS
    Jeban Ganesalingam
    Department of Clinical Neuroscience, King s College London, King s Health Partners, MRC Centre for Neurodegeneration Research, London, UK
    J Neurochem 117:528-37. 2011
    ..These findings support large-scale prospective biomarker studies to determine the clinical utility of diagnostic and prognostic signatures in ALS...
  23. pmc KATP channel subunits in rat dorsal root ganglia: alterations by painful axotomy
    Vasiliki Zoga
    Department of Anesthesiology, Medical College of Wisconsin, Milwaukee, WI 53226, USA
    Mol Pain 6:6. 2010
    ....
  24. ncbi Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy
    Lyndsay M Murray
    Centre for Integrative Physiology, University of Edinburgh Medical School, Edinburgh EH8 9XD, UK
    Hum Mol Genet 17:949-62. 2008
    ....
  25. pmc Neurofilament heavy chain side arm phosphorylation regulates axonal transport of neurofilaments
    Steven Ackerley
    Department of Neuroscience, The Institute of Psychiatry, Denmark Hill, London SE5 8AF, UK
    J Cell Biol 161:489-95. 2003
    ..Thus, phosphorylation of NFH slows neurofilament transport, and this is due to increased pausing in neurofilament movement...
  26. ncbi Charcot-Marie-Tooth disease neurofilament mutations disrupt neurofilament assembly and axonal transport
    Janet Brownlees
    Department of Neuroscience, The Institute of Psychiatry, King s College London, De Crespigny Park, Denmark Hill, London SE5 8AF, UK
    Hum Mol Genet 11:2837-44. 2002
    ..Our results demonstrate that aberrant neurofilament assembly and transport can induce neurological disease, and further implicate defective neurofilament metabolism in the pathogenesis of human neurodegenerative diseases...
  27. ncbi Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS)
    Kathryn Volkening
    Molecular Brain Research Group, Robarts Research Institute, 100 Perth Drive, London, Ontario, Canada N6A 5K8
    Brain Res 1305:168-82. 2009
    ..These data suggest that NFL mRNA processing is fundamentally altered in ALS spinal motor neurons to favour compartmentalization within both stress granules and P-bodies, and that TDP-43 plays a fundamental role in this process...
  28. ncbi A specific ELISA for measuring neurofilament heavy chain phosphoforms
    A Petzold
    Department of Neuroimmunology, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK
    J Immunol Methods 278:179-90. 2003
    ..A new nomenclature is proposed to facilitate the comparison between ELISA, immunoblotting and immunocytochemistry...
  29. ncbi Neurofilament and glial fibrillary acidic protein in multiple sclerosis
    N Norgren
    Department of Immunology, University of Umea, Umea, Sweden
    Neurology 63:1586-90. 2004
    ..To evaluate levels of neurofilament light (NFL) and glial fibrillary acidic protein (GFAP) in CSF from patients with multiple sclerosis (MS) in relation to clinical progress of the disease...
  30. ncbi Rapid movement of axonal neurofilaments interrupted by prolonged pauses
    L Wang
    Neuroscience Programme, Department of Biological Sciences, Ohio University, Athens, Ohio 45701, USA
    Nat Cell Biol 2:137-41. 2000
    ..The movement is bidirectional, but predominantly anterograde. Our data indicate that the slow rate of slow axonal transport may be the result of rapid movements interrupted by prolonged pauses...
  31. ncbi Immunoreactivity of the phosphorylated axonal neurofilament H subunit (pNF-H) in blood of ALS model rodents and ALS patients: evaluation of blood pNF-H as a potential ALS biomarker
    Kevin Boylan
    Department of Neurology, Mayo Clinic, Jacksonville, Florida 32610, USA
    J Neurochem 111:1182-91. 2009
    ..These data suggest that blood pNF-H can be used to monitor axonal degeneration in ALS model rodents and support further study of this protein as a potential biomarker of disease prognosis in ALS patients...
  32. ncbi Mitogen-activated protein kinases (Erk1,2) phosphorylate Lys-Ser-Pro (KSP) repeats in neurofilament proteins NF-H and NF-M
    - Veeranna
    Laboratory of Neurochemistry, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Neurosci 18:4008-21. 1998
    Mammalian neurofilament proteins, particularly midsized (NF-M) and heavy (NF-H) molecular weight neurofilament proteins, are highly phosphorylated in axons...
  33. ncbi Intermediate forms of Charcot-Marie-Tooth neuropathy: a review
    Garth Nicholson
    University of Sydney, The Molecular Medicine and ANZAC Research Institute, Northcott Neuroscience Laboratory, Concord Hospital, NSW 2139, Australia
    Neuromolecular Med 8:123-30. 2006
    ..Intermediate forms of CMT may be associated with unique disease mechanisms affecting both Schwann cells and axons. It is useful to recognize this unique group of neuropathies for diagnostic and management purposes...
  34. ncbi Splice variant-specific interaction of the NMDA receptor subunit NR1 with neuronal intermediate filaments
    M D Ehlers
    Department of Neuroscience, Howard Hughes Medical Institute, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA
    J Neurosci 18:720-30. 1998
    ..These results demonstrate the splice variant-specific association of NR1 with neurofilaments and suggest a possible mechanism for anchoring or localizing NMDA receptors in the neuronal plasma membrane...
  35. ncbi p38alpha stress-activated protein kinase phosphorylates neurofilaments and is associated with neurofilament pathology in amyotrophic lateral sclerosis
    Steven Ackerley
    Departments of Neuroscience and Neurology, The Institute of Psychiatry, Kings College, London, UK
    Mol Cell Neurosci 26:354-64. 2004
    ..Thus, p38 kinases may contribute to the aberrant phosphorylation of NFM and NFH side-arms in ALS...
  36. ncbi Lysolecithin induces demyelination in vitro in a cerebellar slice culture system
    Eric Birgbauer
    Department of Neurobiology, Merck Research Laboratories, San Diego, California, USA
    J Neurosci Res 78:157-66. 2004
    ..Lysolecithin thus induces demyelination in an in vitro organotypic cerebellar slice culture system, providing a model system for studying myelination, demyelination, and remyelination in vitro...
  37. ncbi Areas of cat auditory cortex as defined by neurofilament proteins expressing SMI-32
    Jeffrey G Mellott
    Centre for Brain and Mind, Department of Physiology and Pharmacology, Schulich School of Medicine and Dentistry, The University of Western Ontario, Medical Sciences Building, Room 216, 1151 Richmond Street North, London, Ontario N6A 5C1, Canada
    Hear Res 267:119-36. 2010
    ..SMI-32 labels non-phosphorylated epitopes on the high- and medium-molecular weight subunits of neurofilament proteins in cortical pyramidal cells and dendritic trees with the most robust immunoreactivity in layers III and V...
  38. ncbi Transient blockade of the CD11d/CD18 integrin reduces secondary damage after spinal cord injury, improving sensory, autonomic, and motor function
    Denis Gris
    Spinal Cord Injury Team, BioTherapeutics Research Group, Robarts Research Institute, University of Western Ontario, London, Ontario N6A 5K8, Canada
    J Neurosci 24:4043-51. 2004
    ..This early neuroprotective treatment would be an ideal foundation for building later cell-based therapies...
  39. ncbi Elevated neurofilament levels in neurological diseases
    Niklas Norgren
    Department of Immunology, University of Umea, Umea, Sweden
    Brain Res 987:25-31. 2003
    ..NF-L determinations will be a valuable complement in identifying neuronal degradation and can be used clinically for diagnostic and monitoring purposes...
  40. ncbi Selective loss of trans-acting instability determinants of neurofilament mRNA in amyotrophic lateral sclerosis spinal cord
    Wei Wen Ge
    Cell Biology Research Group, Robarts Research Institute, London, Ontario N6A 5K8, Canada
    J Biol Chem 278:26558-63. 2003
    ..These findings suggest that the trans-acting NFL-destabilizing elements are selectively suppressed in ALS homogenates, resulting in an increased stability and level of NFL mRNA...
  41. pmc Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A
    Chun hong Xia
    Department of Cellular and Molecular Medicine, Howard Hughes Medical Institute, University of California, San Diego, La Jolla, CA 92093 0683, USA
    J Cell Biol 161:55-66. 2003
    ..These data support the hypothesis that a conventional kinesin plays a role in the microtubule-dependent slow axonal transport of at least one cargo, the NF proteins...
  42. pmc Peptidyl-prolyl isomerase 1 regulates protein phosphatase 2A-mediated topographic phosphorylation of neurofilament proteins
    Parvathi Rudrabhatla
    Laboratory of Neurochemistry, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
    J Neurosci 29:14869-80. 2009
    ..This study highlights a novel signaling role of PP2A by Pin1 and implicates Pin1 as a therapeutic target to reduce aberrant phosphorylation of NF proteins in neurodegenerative disorders such as AD, PD, and ALS...
  43. ncbi Alphab-crystallin is a target for adaptive immune responses and a trigger of innate responses in preactive multiple sclerosis lesions
    Johannes M van Noort
    TNO Quality of Life, Leiden, The Netherlands
    J Neuropathol Exp Neurol 69:694-703. 2010
    ..At the same time, however, accumulated CRYAB may represent a major target for adaptive immune responses that could contribute to progression of preactive lesions to a stage of demyelination...
  44. ncbi Expression of neuronal markers suggests heterogeneity of chick sympathoadrenal cells prior to invasion of the adrenal anlagen
    Uwe Ernsberger
    Department of Neuroanatomy, Interdisciplinary Center for Neurosciences IZN, University of Heidelberg, INF 307, 69120 Heidelberg, Germany
    Cell Tissue Res 319:1-13. 2005
    ....
  45. ncbi Axonal damage markers in cerebrospinal fluid are increased in ALS
    J Brettschneider
    Department of Neurology, University of Ulm, Ulm, Germany
    Neurology 66:852-6. 2006
    ..To test whether biomarkers for axonal degeneration correlated with clinical subtypes and were of use in predicting progression of ALS...
  46. ncbi Phosphorylated neurofilament heavy subunit (pNF-H) in peripheral blood and CSF as a potential prognostic biomarker in amyotrophic lateral sclerosis
    Kevin B Boylan
    Department of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA
    J Neurol Neurosurg Psychiatry 84:467-72. 2013
    ..We examined pNF-H concentrations in plasma, serum and CSF as a potential biomarker for disease progression and survival in ALS...
  47. ncbi A systematic review and meta-analysis of CSF neurofilament protein levels as biomarkers in dementia
    A Petzold
    Department of Neuroimmunology, Institute of Neurology, UCL, London, UK
    Neurodegener Dis 4:185-94. 2007
    ..Loss of cortical neurons is a key pathological feature in neurodegenerative dementias. Cerebrospinal fluid (CSF) neurofilaments (Nf) are a biomarker for neuronal death and axonal loss...
  48. ncbi Neurofilaments as biomarkers in multiple sclerosis
    Charlotte E Teunissen
    Department of Clinical Chemistry, VU University Medical Center, The Netherlands
    Mult Scler 18:552-6. 2012
    ..Future studies should be aimed at the optimisation, standardisation and interlaboratory implementation of the assays and address the predictive value of these biomarkers...
  49. ncbi Rho guanine nucleotide exchange factor is an NFL mRNA destabilizing factor that forms cytoplasmic inclusions in amyotrophic lateral sclerosis
    Cristian A Droppelmann
    Molecular Brain Research Group, Robarts Research Institute, University of Western Ontario, London, Ontario, Canada
    Neurobiol Aging 34:248-62. 2013
    ..This is also the first described link between ALS and an RNA binding protein with aggregate formation that is also a central cell signaling pathway molecule...
  50. ncbi Amyloid-related biomarkers and axonal damage proteins in parkinsonian syndromes
    Sara Bech
    Department of Neurology, Bispebjerg Hospital, Copenhagen University Hospital, Copenhagen, Denmark
    Parkinsonism Relat Disord 18:69-72. 2012
    ....
  51. pmc Neurofilament-dependent radial growth of motor axons and axonal organization of neurofilaments does not require the neurofilament heavy subunit (NF-H) or its phosphorylation
    M V Rao
    Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, California 92093, USA
    J Cell Biol 143:171-81. 1998
    ....
  52. pmc Alzheimer disease tangles share immunological similarities with multiphosphorylation repeats in the two large neurofilament proteins
    V M Lee
    Department of Pathology and Laboratory Medicine Neuropathology, University of Pennsylvania School of Medicine, Philadelphia 19104
    Proc Natl Acad Sci U S A 85:7384-8. 1988
    ..We conclude that NFTs share highly specific immunological and structural properties with specific rat heaviest NF subunit and human middle-sized NF subunit multiphosphorylation repeats...
  53. ncbi Evidence for acute neurotoxicity after chemotherapy
    Axel Petzold
    Department of Neuroimmunology, UCL Institute of Neurology, Queen Square, London, United Kingdom
    Ann Neurol 68:806-15. 2010
    ..Neurotoxicity adversely affects patients' quality of life. The objective of this study is to examine whether there is evidence of acute neurotoxicity...
  54. pmc Gene replacement in mice reveals that the heavily phosphorylated tail of neurofilament heavy subunit does not affect axonal caliber or the transit of cargoes in slow axonal transport
    Mala V Rao
    Nathan Kline Institute, New York University School of Medicine, Orangeburg, NY 10962, USA
    J Cell Biol 158:681-93. 2002
    ..Most surprisingly, at least in optic nerve axons, loss of the NF-H tail does not affect the rate of transport of neurofilament subunits...
  55. ncbi Phosphorylation of the high molecular weight neurofilament protein (NF-H) by Cdk5 and p35
    D Sun
    Department of Pathology, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    J Biol Chem 271:14245-51. 1996
    ..By immunoprecipitating Cdk5 and p35 from the transfected cells, we have been able to show that the KSPXK repeats are the preferred phosphorylation sites for Cdk5, while the KSPXY repeats are not directly phosphorylated by Cdk5 and p35...
  56. ncbi Neurofilament 200 as an indicator of differences between mipafox and paraoxon sensitivity in Sy5Y neuroblastoma cells
    Taehyeon Cho
    Department of Veterinary Anatomy and Public Health and Faculty of Toxicology, Texas A and M University, College Station, Texas 77843 4458, USA
    J Toxicol Environ Health A 67:987-1000. 2004
    ..These results indicate an alternative approach and test system for investigating structure-activity relationships of OPs...
  57. ncbi Identification of Ser-55 as a major protein kinase A phosphorylation site on the 70-kDa subunit of neurofilaments. Early turnover during axonal transport
    R K Sihag
    Laboratory for Molecular Neuroscience, McLean Hospital, Belmont, Massachusetts 02178
    J Biol Chem 266:18861-7. 1991
    ....
  58. ncbi Architectonic subdivision of the human orbital and medial prefrontal cortex
    Dost Ongur
    Massachusetts General Hospital, Boston, Massachusetts 02114, USA
    J Comp Neurol 460:425-49. 2003
    ..The parallel organization of the OMPFC in monkeys and humans allows experimental data from monkeys to be applied to studies of the human cortex...
  59. pmc The myosin Va head domain binds to the neurofilament-L rod and modulates endoplasmic reticulum (ER) content and distribution within axons
    Mala V Rao
    Nathan Kline Institute, New York University School of Medicine, Orangeburg, New York, United States of America
    PLoS ONE 6:e17087. 2011
    ..Based on observations that the Myo Va motor domain binds to intermediate filament (IF) proteins of several classes, Myo Va interactions with IFs may serve similar roles in organizing organelle topography in different cell types...
  60. ncbi Immunohistochemical distinction between merkel cell carcinoma and small cell carcinoma of the lung
    Mattheos Bobos
    Department of Pathology, Aristotle University Medical School, Thessaloniki, Greece
    Am J Dermatopathol 28:99-104. 2006
    ..These findings suggest that a set of three immunohistochemical stains, including CK20, NF, and TTF-1, is useful in affording a distinction between Merkel cell carcinoma and small cell lung carcinoma...
  61. pmc NF-M is an essential target for the myelin-directed "outside-in" signaling cascade that mediates radial axonal growth
    Michael L Garcia
    Ludwig Institute for Cancer Research, Department of Cellular and Molecular Medicine, University of California at San Diego, La Jolla, CA 92093, USA
    J Cell Biol 163:1011-20. 2003
    ..This has revealed that the tail domain of NF-M, with seven KSP motifs, is an essential target for the myelination-dependent outside-in signaling cascade that determines axonal caliber and conduction velocity of motor axons...
  62. ncbi Cerebrospinal fluid neurofilament light levels in amyotrophic lateral sclerosis: impact of SOD1 genotype
    H Zetterberg
    Department of Neurochemistry and Psychiatry, Sahlgrenska University Hospital, Goteborg University, Goteborg, Sweden
    Eur J Neurol 14:1329-33. 2007
    ..518, P = 0.001). NF-L levels were lower in SOD1 mutation-associated ALS compared with SOD1 wild-type (wt) ALS (P = 0.03). In conclusion, CSF NF-L levels may provide both diagnostic and prognostic information, particularly in SOD1 wt ALS...
  63. ncbi A mutant neurofilament subunit causes massive, selective motor neuron death: implications for the pathogenesis of human motor neuron disease
    M K Lee
    Department of Biological Chemistry, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205
    Neuron 13:975-88. 1994
    ..Thus, neurofilament mutations can cause selective motor neuron death, and neurofilamentous abnormalities may be a common toxic intermediate that significantly contributes to the motor neuron death in human disease...
  64. ncbi Astrocyte-specific but not neuron-specific transmembrane TNF triggers inflammation and degeneration in the central nervous system of transgenic mice
    K Akassoglou
    Department of Molecular Genetics, Hellenic Pasteur Institute, Athens, Greece
    J Immunol 158:438-45. 1997
    ..These results demonstrate that target cells mediating the neuroinflammatory activities of TNF localize in the vicinity of astrocytes rather than neurons...
  65. pmc Phosphorylation and compactness of neurofilaments in multiple sclerosis: indicators of axonal pathology
    Axel Petzold
    Department of Neuroinflammation, Institute of Neurology, Queen Square, London, UK
    Exp Neurol 213:326-35. 2008
    ..This study investigated whether neurofilament (NfH) phosphorylation would relate to the dynamics of axonal pathology in multiple sclerosis (MS)...
  66. pmc Dysfunctions of neuronal and glial intermediate filaments in disease
    Ronald K H Liem
    Department of Pathology and Cell Biology, Taub Institute for Research on Alzheimer s Disease and the Aging Brain, Columbia University College of Physicians and Surgeons, New York, NY, USA
    J Clin Invest 119:1814-24. 2009
    ..In this Review, we focus on the IFs of glial cells (primarily GFAP) and neurons as well as their relationship to different neurodegenerative diseases...
  67. ncbi Selective loss of neurofilament expression in Cu/Zn superoxide dismutase (SOD1) linked amyotrophic lateral sclerosis
    Fiona M Menzies
    Academic Neurology Unit and Academic Unit of Pathology, The Medical School, University of Sheffield, South Yorkshire, UK
    J Neurochem 82:1118-28. 2002
    ..These data suggest that NFL mRNA reductions are common to SALS and FALS patients, and that cells and mice expressing mutant SOD1 may enable us to characterize the molecular mechanism(s) responsible for the loss of neurofilament mRNA...
  68. ncbi Mutant copper-zinc superoxide dismutase binds to and destabilizes human low molecular weight neurofilament mRNA
    Wei Wen Ge
    Cell Biology Research Group, Robarts Research Institute, London, Ontario N6A 5K8, Canada
    J Biol Chem 280:118-24. 2005
    ..These observations provide an explanation for a novel gain of function in which mutant SOD1 expression in motor neurons alters an intermediate filament protein expression...
  69. ncbi Amyloid beta accumulation in axons after traumatic brain injury in humans
    Douglas H Smith
    Department of Neurosurgery, University of Pennsylvania, Philadelphia, Pennsylvania 19104 6316, USA
    J Neurosurg 98:1072-7. 2003
    ..In the present study, the authors explored the potential relationship between Abeta accumulation in damaged axons and associated Abeta plaque formation...
  70. ncbi Post-traumatic hypoxia exacerbates brain tissue damage: analysis of axonal injury and glial responses
    Sarah C Hellewell
    National Trauma Research Institute, The Alfred Hospital, Melbourne, Victoria, Australia
    J Neurotrauma 27:1997-2010. 2010
    ....
  71. ncbi Acute axonal damage predicts clinical outcome in patients with multiple sclerosis
    E T Lim
    Department of Neuroinflammation, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, UK
    Mult Scler 11:532-6. 2005
    ..This study supports the view that acute inflammation in ON and MS results in axonal pathology and that the latter has a role in determining functional impairment...
  72. ncbi Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: a mouse model of amyotrophic lateral sclerosis
    F Cote
    Centre for Research in Neurosciences McGill University, Canada
    Cell 73:35-46. 1993
    ....
  73. pmc Neurofilament subunit NF-H modulates axonal diameter by selectively slowing neurofilament transport
    J R Marszalek
    Division of Cellular and Molecular Medicine, University of California at San Diego, La Jolla 92093, USA
    J Cell Biol 135:711-24. 1996
    ....
  74. pmc Altered axonal architecture by removal of the heavily phosphorylated neurofilament tail domains strongly slows superoxide dismutase 1 mutant-mediated ALS
    Christian S Lobsiger
    Ludwig Institute for Cancer Research and Department of Medicine, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093, USA
    Proc Natl Acad Sci U S A 102:10351-6. 2005
    ....
  75. ncbi Alpha-internexin is structurally and functionally associated with the neurofilament triplet proteins in the mature CNS
    Aidong Yuan
    Center for Dementia Research, Nathan Kline Institute, Orangeburg, New York 10962, USA
    J Neurosci 26:10006-19. 2006
    ..Alpha-internexin also coassembles with all three neurofilament proteins into a single network of filaments in quadruple-transfected SW13vim(-) cells...
  76. ncbi Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease
    Z Xu
    Department of Biological Chemistry, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205
    Cell 73:23-33. 1993
    ..These data indicate that extensive accumulation of neurofilaments in motor neurons can trigger the neurodegenerative process...
  77. ncbi A NUDEL-dependent mechanism of neurofilament assembly regulates the integrity of CNS neurons
    Minh Dang Nguyen
    Department of Pathology, Harvard Medical School and Howard Hughes Medical Institute, 77 Avenue Louis Pasteur, New Research Building, Room 856 8, MA 02115, USA
    Nat Cell Biol 6:595-608. 2004
    ..Furthermore, variations in levels of NUDEL correlate with disease progression and NF defects in a mouse model of neurodegeneration. Thus, NUDEL contributes to the integrity of CNS neurons by regulating NF assembly...
  78. ncbi Neurofilaments and neurological disease
    Ammar Al-Chalabi
    Departments of Neuroscience and Neurology, Institute of Psychiatry, King s College London, London SE5 8AF, UK
    Bioessays 25:346-55. 2003
    ..In this review, we discuss the structure, normal function and molecular pathology of neurofilaments...
  79. ncbi Myelin-associated glycoprotein is a myelin signal that modulates the caliber of myelinated axons
    X Yin
    Department of Neurosciences, Lerner Research Institute, The Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA
    J Neurosci 18:1953-62. 1998
    ....
  80. ncbi Patients with amyotrophic lateral sclerosis and other neurodegenerative diseases have increased levels of neurofilament protein in CSF
    L E Rosengren
    Institute of Anatomy and Cell Biology, University of Goteborg, Sweden
    J Neurochem 67:2013-8. 1996
    ..0001). NFL is a main structural protein of axons, and we suggest that CSF NFL can be used to monitor neurodegeneration in general, but particularly in ALS with involvement of the pyramidal tract...
  81. ncbi High molecular weight neurofilament proteins are physiological substrates of adduction by the lipid peroxidation product hydroxynonenal
    Takafumi Wataya
    Institute of Pathology and Departments of Physiology and Biophysics, and Chemistry, Case Western Reserve University, Cleveland, Ohio 44106, USA
    J Biol Chem 277:4644-8. 2002
    ....
  82. ncbi Disruption of dynein/dynactin inhibits axonal transport in motor neurons causing late-onset progressive degeneration
    Bernadette H LaMonte
    Department of Physiology, School of Medicine, University of Pennsylvania, 3700 Hamilton Walk, Philadelphia 19104, USA
    Neuron 34:715-27. 2002
    ..In this report, we describe a mouse model that confirms the critical role of disrupted axonal transport in the pathogenesis of motor neuron degenerative disease...
  83. pmc Tight functional coupling of kinesin-1A and dynein motors in the bidirectional transport of neurofilaments
    Atsuko Uchida
    Center for Molecular Neurobiology and Department of Neuroscience, The Ohio State University, Columbus, OH 43210, USA
    Mol Biol Cell 20:4997-5006. 2009
    ....
  84. pmc Axonal degeneration and inflammation in acute optic neuritis
    A Petzold
    Department of Neuroinflammation, Institute of Neurology, University College London, Queen Square, UK
    J Neurol Neurosurg Psychiatry 75:1178-80. 2004
    ..To investigate whether plasma biomarkers for axonal injury and inflammation are related to loss and recovery of visual function in acute optic neuritis (ON)...
  85. ncbi A method to solubilise protein aggregates for immunoassay quantification which overcomes the neurofilament "hook" effect
    Ching Hua Lu
    Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, University College London, London WC1N3BG, UK
    J Neurosci Methods 195:143-50. 2011
    ..Using the SOD1(G93A) mouse model of amyotrophic lateral sclerosis, we developed a method to disrupt Nf aggregates, allowing optimal immunoassay performance...
  86. ncbi Conditional NF-L transgene expression in mice for in vivo analysis of turnover and transport rate of neurofilaments
    Stephanie Millecamps
    Centre de Recherche du Centre Hospitalier de l Universite Laval, Department of Anatomy and Physiology of Laval University, Quebec, Canada
    J Neurosci 27:4947-56. 2007
    ..These results provide in vivo evidence that the stationary NF network in axons is a key determinant of half-life and transport rate of NF proteins...
  87. ncbi Neurofilament ELISA validation
    Axel Petzold
    Department of Neuroinflammation, UCL Institute of Neurology, Queen Square, London WC1N 3BG, United Kingdom
    J Immunol Methods 352:23-31. 2010
    b>Neurofilament proteins (Nf) are highly specific biomarkers for neuronal death and axonal degeneration...
  88. pmc Shank 2 expression coincides with neuronal differentiation in the developing retina
    Jeong Hun Kim
    Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul 110 744, Korea
    Exp Mol Med 41:236-42. 2009
    ..In conclusion, our data suggests that Shank 2 is expressed in the neurons of the developing retina and could play a critical role in the neuronal differentiation of the developing retina...
  89. ncbi In vivo and in vitro phosphorylation at Ser-493 in the glutamate (E)-segment of neurofilament-H subunit by glycogen synthase kinase 3beta
    Takahiro Sasaki
    Department of Biological Sciences, Graduate School of Science, Tokyo Metropolitan University, Minami Ohsawa, Hachiohji, Tokyo 192 0397, Japan
    J Biol Chem 277:36032-9. 2002
    ..GSK3beta in the spinal cord extract was associated with NF cytoskeletons. Taken together, we concluded that Ser-493 in the E-segment of NF-H is phosphorylated by GSK3beta in rat spinal cords...
  90. ncbi Adult rat and human bone marrow stromal cells differentiate into neurons
    D Woodbury
    Department of Neuroscience and Cell Biology, UMDNJ Robert Wood Johnson Medical School, Piscataway, New Jersey 08854, USA
    J Neurosci Res 61:364-70. 2000
    ..Consequently, adult marrow stromal cells can be induced to overcome their mesenchymal commitment and may constitute an abundant and accessible cellular reservoir for the treatment of a variety of neurologic diseases...
  91. ncbi Review of the multiple aspects of neurofilament functions, and their possible contribution to neurodegeneration
    Rodolphe Perrot
    Centre de Recherche du Centre Hospitalier Universitaire de Québec, Department of Anatomy and Physiology of Laval University, Quebec, G1V 4G2, Canada
    Mol Neurobiol 38:27-65. 2008
    ..However, despite the presence of NF mutations found in some patients, the exact relations between these mutations, the abnormal NF organisation and the pathological process remain a challenging field of investigation...
  92. ncbi Attenuated neurodegenerative disease phenotype in tau transgenic mouse lacking neurofilaments
    T Ishihara
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Neurosci 21:6026-35. 2001
    ....
  93. ncbi Peripherin and neurofilaments: expression and role during neural development
    M M Portier
    College de France, Laboratoire de Biochimie Cellulaire, Paris, France
    C R Acad Sci III 316:1124-40. 1993
    ..in neurons but exclusively in motor, sensory and sympathetic neurons; moreover, it is co-expressed with neurofilament proteins (NFP)...
  94. ncbi Neuronal differentiation of murine bone marrow Thy-1- and Sca-1-positive cells
    F Locatelli
    Centro Dino Ferrari, Dipartimento di Scienze Neurologiche, Universita degli Studi di Milano, I R C C S Ospedale Maggiore Policlinico, Milan, Italy
    J Hematother Stem Cell Res 12:727-34. 2003
    ..Although cell fusion phenomena with the host cells could not be ruled out, bone marrow-derived neurosphere transplantation could be a strategy for cellular mediated gene therapy...
  95. ncbi Identification of six phosphorylation sites in the COOH-terminal tail region of the rat neurofilament protein M
    Z S Xu
    Department of Anatomy and Neurobiology and Biochemistry, Washington University School of Medicine, St Louis, Missouri 63110
    J Biol Chem 267:4467-71. 1992
    ..The identification of these sites should aid in investigations of the function of the phosphorylation of this protein and provides criteria for identifying the relevant kinases...
  96. ncbi Distribution of plectin, an intermediate filament-associated protein, in the adult rat central nervous system
    L D Errante
    Department of Neuroscience, University of Florida College of Medicine, Gainesville 32610 0244
    J Neurosci Res 37:515-28. 1994
    ....
  97. ncbi Neurofilaments in health and disease
    T Gotow
    Laboratory of Cell Biology, College of Nutrition, Koshien University, 10 1 Momijigaoka, Takarazuka 665 0006, Japan
    Med Electron Microsc 33:173-99. 2000
    ....
  98. ncbi Defective neurofilament transport in mouse models of amyotrophic lateral sclerosis: a review
    Mala V Rao
    Center for Dementia Research, Nathan Kline Institute Department of Psychiatry, NYU School of Medicine, 140 Old Orangeburg Road, Orangeburg, New York 10962, USA
    Neurochem Res 28:1041-7. 2003
    b>Neurofilament proteins synthesized in the cell body of neurons are assembled and transported into axons, where they influence axon radial growth, axonal transport, and nerve conduction velocities...
  99. ncbi Dynamic behavior and organization of cytoskeletal proteins in neurons: reconciling old and new findings
    R A Nixon
    Nathan Kline Institute, New York University Medical Center, Orangeburg 10962, USA
    Bioessays 20:798-807. 1998
    ..This regulation involves, in part, a system of protein kinases and phosphatases modulated by both intercellular and intracellular signals. Conceptual models of slow axonal transport have evolved to accommodate these new findings...
  100. ncbi Quantitative ultrastructural analysis of the neurofilament 200-positive axons in the rat dental pulp
    Sang Kyoo Paik
    Department of Oral Anatomy and Neurobiology, School of Dentistry, Kyungpook National University, Daegu, Korea
    J Endod 36:1638-42. 2010
    ..In this study, we investigated the morphologic changes of pulpal myelinated axons between their root portion (radicular pulp) and their terminal area (peripheral pulp)...
  101. ncbi Progressive changes in neurofilament proteins and growth-associated protein-43 immunoreactivities at the site of cervical spinal cord compression in spinal hyperostotic mice
    Kenzo Uchida
    Department of Orthopaedic Surgery, School of Medicine, Fukui Medical University, Matsuoka, Fukui, Japan
    Spine (Phila Pa 1976) 27:480-6. 2002
    ..Immunohistochemical examination of the expression and localization of neurofilament (NF) proteins and growth-associated protein (GAP)-43 in spinal hyperostotic (twy/twy) mice with progressive compression of the cervical spinal cord...

Research Grants62

  1. Dysfunction of neurofilaments in disease: an iPS model of Giant Axonal Neuropathy
    BETHANY LESLIE JOHNSON-KERNER; Fiscal Year: 2012
    ..Biopsies of GAN patients are characterized by the presence of accumulations of neurofilament proteins (NFs) in neurons and intermediate filaments (IFs) in a variety of other cell types, including astrocytes ..
  2. Pathogenesis Of Essential Tremor: Cerebellar Metabolism
    Elan D Louis; Fiscal Year: 2012
    ..In Aim 1A, we will also begin to study neurofilament proteins. Because our previous analyses were confined to a single region of one cerebellar hemisphere, we will ..
  3. MOLECULAR MARKERS OF PROGNOSIS IN MEDULLOBLASTOMA
    SANDRA BIGNER; Fiscal Year: 2000
    ..and will define, by immunohistochemistry, the distribution of synaptophysin, neuron-specific enolase, neurofilament proteins, and glial fibrillary acidic protein, in these specimens...
  4. CALMODULIN DEPENDENT KINASE AND SYNAPTIC FUNCTION
    MARY VALLANO; Fiscal Year: 1992
    ..Phosphorylation of neurofilament proteins by CaM kinase II may stabilize neurofilaments...
  5. MECHANISMS OF OCCUPATIONAL NEUROPATHIES
    MOHAMED ABOU DONIA; Fiscal Year: 1999
    ..recent findings of the increase in CaM kinase II mRNA expression and slow axonal transport of radiolabeled neurofilament proteins in OPIDN...
  6. FAMILIAL ALS--MECHANISM OF INITIATION
    Dale Bredesen; Fiscal Year: 1999
    ..and functionally evaluating 1) glutamate transporters (Volterra lab, oxidative modification), 2) neurofilament proteins (Cleveland lab) and structurally only with 3) membrane lipids (Bredesen lab, lipid peroxides)...
  7. INTERMEDIATE FILAMENTS--MOLECULAR APPROACH TO FUNCTION
    STEPHEN TAPSCOTT; Fiscal Year: 1990
    ..g., desmin in inherited myopathies, neurofilament proteins and vimentin in the neurofibrillary tangles of Alzheimer's disease, and neurofilaments in motor neuron ..
  8. Calpain Inhibition Strategies for Spinal Cord Injury
    JAMES GEDDES; Fiscal Year: 2007
    ..In previous studies, postinjury administration of calpain inhibitors attenuated axonal damage and loss of neurofilament proteins, but functional outcome was not examined and the magnitude of calpain inhibition was not measured...
  9. MECHANISMS OF DYING BACK NEUROPATHIES
    MOHAMED ABOU DONIA; Fiscal Year: 1993
    ..The mechanisms through which these alterations occur involve a direct binding of the toxicant to neurofilament proteins, an inhibition of protein phosphorylation, an inhibition of proteolytic proteins...
  10. TOXIC MODELS OF NEUROFIBRILLARY PATHOLOGY
    Douglas Anthony; Fiscal Year: 1991
    ..This covalent crosslinking of neurofilament proteins is proposed to be a cumulative process that ultimately results in neurofilamentous tangles which preclude ..
  11. DYNAMICS OF THE NEURONAL CYTOSKELETON IN AGING BRAIN
    Ralph Nixon; Fiscal Year: 2000
    ..will be investigated in studies on the early assembly events, phosphorylation, and turnover of neurofilament proteins and tubulin in young adult and aged mice...
  12. Novel Technology for the Detection of Low Abundance Protein
    Cha Mei Tang; Fiscal Year: 2009
    ..In Phase I of this SBIR, we plan to recover human neurofilament proteins from serum...
  13. ZINC DEFICIENT SOD AND NEUROFILAMENT DYSFUNCTION IN ALS
    John Crow; Fiscal Year: 2001
    ..Lastly, peroxynitrite treatment assembly of triplet neurofilament proteins in vitro, presumably by introducing a permanent negative charge on hydrophobic tyrosine...
  14. CSF AND PLASMA NEUROFILAMENTS IN TRAUMATIC BRAIN INJURY
    Frank Zemlan; Fiscal Year: 1999
    ..The proposed laboratory test will quantify levels of neurofilament proteins. Neurofilament proteins are neuron- specific proteins localized primarily in the axonal compartment...
  15. MECHANISMS OF ACTION OF SMALL-MOLECULE NEUROTOXINS
    LAWRENCE SAYRE; Fiscal Year: 1991
    ..clarify the structural basis of toxicity, particularly in respect to the direct chemical modification of neurofilament proteins by the neurotoxic chemical (or metabolite hereof)...
  16. NEUROFIBRILLARY PATHOLOGY IN ALZHEIMER DISEASE
    George Perry; Fiscal Year: 1999
    ..Further suggestions of this come from monoclonal antibodies raised to NFT which recognize AGE adducts of neurofilament proteins as well as t. The latter, Alz50, which recognizes a reducible (i.e...
  17. NEUROFILAMENTS--BIOCHEMISTRY AND ASSEMBLY
    FUNG CHOW CHIU; Fiscal Year: 1992
    ..We will measure reassembly of iodinated neurofilament proteins to proteins that are bound to nitrocellulose sheets.
  18. CALCIUM AND THE CYTOSKELETON IN ALZHEIMER'S
    CAROL TROY; Fiscal Year: 1993
    ..The role of peripherin in neurological diseases will be investigated in model systems for neurodegenerative disorders and in human neuropathological specimens of motor neuron disease...
  19. Nanospheres as Vehicles for Treatment of Neuroblastoma
    Thomas Shea; Fiscal Year: 2004
    ..Resulting data will indicate whether or not nanospheres represent useful delivery agents for cancer therapy. ..
  20. Intermediate Filaments 2008 Gordon Research Conference
    Thomas Shea; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable]..
  21. Is S-adenosyl Methionine a Nutrition: Genetic Link in AD?
    Thomas Shea; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable]..
  22. Methylphenidate & gene expression in the rat brain.
    JOAN BAIZER; Fiscal Year: 2004
    ..abstract_text> ..
  23. MOLECULAR MECHANISMS OF HEXACARBON-INDUCED AXON ATROPHY
    RICHARD LOPACHIN; Fiscal Year: 2009
    ..Understanding the role and mechanism of axon atrophy in solvent neurotoxicity has broad-based implications for human occupational health and risk assessment. ..
  24. Medial Pain Inputs to Monkey Anterior Cingulate Cortex
    Brent Vogt; Fiscal Year: 2008
    ..abstract_text> ..
  25. Reverse genetics using RNAi in non-mouse mammals
    Zuoshang Xu; Fiscal Year: 2008
    ..unreadable] [unreadable] [unreadable] [unreadable] [unreadable]..
  26. Silencing mutant SOD1 in vivo for treatment of ALS
    Zuoshang Xu; Fiscal Year: 2008
    ..If successful, these experiments will provide impetus for further human trials of this therapeutic strategy. [unreadable] [unreadable] [unreadable]..
  27. Engineering Fusogenic properties of lipid bilayers
    Mark Stevens; Fiscal Year: 2007
    ..That in turn will significantly advance the utility of liposomes as drug delivery vehicles, and contribute important insights to biological processes where fusion is involved. [unreadable] [unreadable] [unreadable]..
  28. Modeling Progranulin hypomorphism for FTD in mice
    Zuoshang Xu; Fiscal Year: 2008
    ..If successful, our work will establish the first animal model for this disease, which can be used for studying neurodegeneration mechanisms and testing therapeutic strategies. [unreadable] [unreadable] [unreadable]..
  29. Spinal cord repair with nerve constructs
    Douglas Smith; Fiscal Year: 2009
    ..abstract_text> ..
  30. Surrogate markers for brain damage
    Robert Siman; Fiscal Year: 2009
    ....
  31. MRI of Angiogenesis in Spinal Cord Injury
    Ponnada Narayana; Fiscal Year: 2009
    ..In addition, it is possible to modulate angiogenesis in conjunction with other treatments, such as cellular grafts to further enhance the recovery from SCI. Thus the proposed studies have a very high degree of clinical relevance. ..
  32. Attenuation of Axonal Damage and Neuronal Death in EAE
    Naren L Banik; Fiscal Year: 2010
    ..abstract_text> ..
  33. Understanding mechanism and therapy of ALS using RNAi
    Zuoshang Xu; Fiscal Year: 2008
    ..To determine the optimal time for therapy, we will use the Tamoxifeninducible Cre recombinant system to determine at what stage of the disease induction of shRNA to suppress mutant SOD1expresion is most effective. ..
  34. MECHANISMS OF NO-MEDIATED PROTEIN S-NITROSYLATION IN EAE
    OSCAR BIZZOZERO; Fiscal Year: 2007
    ..abstract_text> ..
  35. ESTROGEN EFFICACY IN SPINAL CORD INJURY
    Naren Banik; Fiscal Year: 2006
    ..Understanding estrogen's mechanisms in cell protection and recovery as it relates to multidestructive pathways will establish it as a viable SCI treatment agent. ..
  36. MAGNETIC RESONANCE OF SPINAL CORD INJURY
    Ponnada Narayana; Fiscal Year: 2006
    ..With recent advances in the treatment of SCI, there is a critical need to noninvasively monitor the efficacy of a given treatment. The proposed MRI and fMRI studies greatly aid realize this goal. ..
  37. NITRIC OXIDE, PLP ACYLATION & THE PATHOPHYSIOLOGY OF MS
    OSCAR BIZZOZERO; Fiscal Year: 2003
    ..abstract_text> ..
  38. CALPAIN IN THE PATHOPHYSIOLOGY OF DEMYELINATING DISEASE
    Naren Banik; Fiscal Year: 2003
    ....
  39. 3 T Whole Body Magnetic Resonance Scanner
    Ponnada Narayana; Fiscal Year: 2004
    ..Strong institutional commitment and financial plans for operating the scanner, particularly the long-term maintenance, are described. ..
  40. BRAIN INJURY, AGING AND NEURODEGENERATION IN THE RAT
    Douglas Smith; Fiscal Year: 2004
    ..Success of these studies may reveal mechanistic links between brain trauma, age and neurodegenerative processes and provide a basis for the development of new therapeutic strategies. ..
  41. CALPAIN ACTIVATION OF T CELLS IN DEMYELINATING DISEASE
    Naren Banik; Fiscal Year: 2009
    ..Understanding the mechanisms may help develop therapeutic strategies (e.g., calpain as a target) for treatment of MS. ..
  42. Developing animal models for diseases using RNAi
    Zuoshang Xu; Fiscal Year: 2005
    ..If successful, this technology can be widely applied as an alternative to gene knockout technology for investigation of gene functions in vivo and generation of animal disease models. ..
  43. SERIAL MAGNETIC RESONANCE IN MULTIPLE SCLEROSIS
    Ponnada Narayana; Fiscal Year: 2002
    ..A further significance of these studies is that they may enable us to follow objectively and quantitatively the efficacy of drugs in the treatment of MS. ..
  44. MULTIFOCAL NEURODEGENERATION IN ALZHEIMER'S DISEASE
    Brent Vogt; Fiscal Year: 2002
    ....
  45. High Field MRI Scanner for Animal Studies
    Ponnada Narayana; Fiscal Year: 2003
    ..Organizational and financial plans for administering and maintaining the scanner are described. To the best of the PI's knowledge, this will be the only facility of its kind in Houston and the Southwest part of the USA. ..
  46. DISEASE PROGRESSION AND OXIDATIVE STRESS IN ALS
    Zuoshang Xu; Fiscal Year: 2002
    ..Further, we will test whether and how anti-oxidant treatment changes the course of disease progression. ..
  47. MR Image Analysis in MS: Identification of a Surrogate
    Ponnada Narayana; Fiscal Year: 2002
    ..While the main emphasis is on MS, this system should be readily adaptable to investigate and manage various neurological disorders that require accurate determination of tissue volumes and their temporal change. ..
  48. Mitochondrial dysfunction in ALS
    Zuoshang Xu; Fiscal Year: 2003
    ..A direct association of mutant SOD1 with mitochondria will suggest that mitochondria are damaged directly by the toxicity of mutant enzyme. ..
  49. Bladder Pain Gene Therapy with Pro-opiomelanocortin cDNA
    Naoki Yoshimura; Fiscal Year: 2004
    ..This research project is important to provide a solid basis for potential future clinical application. ..
  50. Spinal Opioid Release in Nociception
    Juan Carlos Marvizon; Fiscal Year: 2008
    ..The neural pathways involved will be studied using selective spinal cord lesions. ..
  51. Synaptic Plasticity and Extinction of Fear
    Kerry Ressler; Fiscal Year: 2007
    ..Emory University and the Center for Behavioral Neuroscience provide an excellent environment for career development and training in the field of behavioral neuroscience. ..
  52. Dopaminergic Influences on Brain Formation and Function
    GREGG STANWOOD; Fiscal Year: 2006
    ..abstract_text> ..
  53. Mechanism of neurodegeneration in dystonia musculorum
    RONALD LIEM; Fiscal Year: 2007
    ..abstract_text> ..
  54. BIOCHEMISTRY OF THE NEURONAL CYTOSKELETON
    RONALD LIEM; Fiscal Year: 2007
    ..We hope that these transgenic models will give us a better indication of how the disease progresses and they should also give us the best system to study the downstream effects of the mutations. ..
  55. CHARACTERIZATION OF TSC PROTEIN HAMARTIN AND TUBERIN
    Vijaya Ramesh; Fiscal Year: 2005
    ..The information obtained here will elucidate the physiological functions of these tumor suppressors, which will aid in designing better therapies. ..
  56. NR3 Expression in Human Brain
    Helena Mueller; Fiscal Year: 2003
    ..abstract_text> ..
  57. In Vivo Studies of TSC-Related Tumorigenesis
    Raymond Yeung; Fiscal Year: 2008
    ..Our long-term goal is to exploit the mechanisms of these pathways in the development of clinical therapy for TSC-related disorders. ..
  58. The Optic Nerve Head in Glaucoma
    Elaine Johnson; Fiscal Year: 2008
    ..abstract_text> ..
  59. TAU ISOFORMS IN HUMAN BRAIN DISEASE AND TRANSGENIC MICE
    Peter Davies; Fiscal Year: 2001
    ....