neurodegenerative diseases

Summary

Summary: Hereditary and sporadic conditions which are characterized by progressive nervous system dysfunction. These disorders are often associated with atrophy of the affected central or peripheral nervous system structures.

Top Publications

  1. pmc Autophagy gone awry in neurodegenerative diseases
    Esther Wong
    Department of Developmental and Molecular Biology, Marion Bessin Liver Research Center and Institute for Aging Studies, Albert Einstein College of Medicine, Bronx, New York, USA
    Nat Neurosci 13:805-11. 2010
  2. ncbi Protein misfolding, functional amyloid, and human disease
    Fabrizio Chiti
    Dipartimento di Scienze Biochimiche, Universita degli Studi di Firenze, I 50134 Firenze, Italy
    Annu Rev Biochem 75:333-66. 2006
  3. pmc Mechanisms underlying inflammation in neurodegeneration
    Christopher K Glass
    Department of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, 92093, USA
    Cell 140:918-34. 2010
  4. ncbi HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS
    Udai Bhan Pandey
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Nature 447:859-63. 2007
  5. ncbi Microglia and inflammation-mediated neurodegeneration: multiple triggers with a common mechanism
    Michelle L Block
    Neuropharmacology Section, MD F1 01, National Institute of Environmental Health Sciences, P O Box 12233, Research Triangle Park, NC 27709, USA
    Prog Neurobiol 76:77-98. 2005
  6. ncbi Microglia-mediated neurotoxicity: uncovering the molecular mechanisms
    Michelle L Block
    Neuropharmacology Section, National Institute of Environmental Health Sciences, Research Triangle Park, North Carolina 27709, USA
    Nat Rev Neurosci 8:57-69. 2007
  7. pmc Self-propagation of pathogenic protein aggregates in neurodegenerative diseases
    Mathias Jucker
    Department of Cellular Neurology, Hertie Institute for Clinical Brain Research, University of Tubingen, D 72076 Tubingen, Germany
    Nature 501:45-51. 2013
  8. ncbi Oxidative stress and neurodegeneration: where are we now?
    Barry Halliwell
    Department of Biochemistry, Yong Loo Lin School of Medicine, National University of Singapore, Singapore
    J Neurochem 97:1634-58. 2006
  9. pmc Mitochondrial dynamics--fusion, fission, movement, and mitophagy--in neurodegenerative diseases
    Hsiuchen Chen
    Division of Biology and Howard Hughes Medical Institute, California Institute of Technology, Pasadena, CA 91125, USA
    Hum Mol Genet 18:R169-76. 2009
  10. pmc The seeds of neurodegeneration: prion-like spreading in ALS
    Magdalini Polymenidou
    Ludwig Institute for Cancer Research and Department of Cellular and Molecular Medicine, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093 0670, USA
    Cell 147:498-508. 2011

Research Grants

  1. NIH Director's Pioneer Award
    Junying Yuan; Fiscal Year: 2009
  2. Enhanced iron removal with peptide linked chelators targeted to ferritin
    Elizabeth C Theil; Fiscal Year: 2010
  3. Gamma-Synuclein-Mediated Regulation of Norepinephrine Transporter
    Anita Sidhu; Fiscal Year: 2010
  4. Sphingolipid Metabolism in Drosophila Development
    Julie D Saba; Fiscal Year: 2013
  5. Samuel D Crish; Fiscal Year: 2016
  6. Inderjit Singh; Fiscal Year: 2014
  7. DHAN V KALVAKOLANU; Fiscal Year: 2014
  8. Monitoring cellular redox signaling and oxidant stress in vivo
    Paul T Schumacker; Fiscal Year: 2010
  9. Enzymatic Regulation of S-Glutathionylation : Role in Apoptosis and Disease
    John J Mieyal; Fiscal Year: 2012
  10. MARK L DELL'ACQUA; Fiscal Year: 2016

Detail Information

Publications424 found, 100 shown here

  1. pmc Autophagy gone awry in neurodegenerative diseases
    Esther Wong
    Department of Developmental and Molecular Biology, Marion Bessin Liver Research Center and Institute for Aging Studies, Albert Einstein College of Medicine, Bronx, New York, USA
    Nat Neurosci 13:805-11. 2010
    ....
  2. ncbi Protein misfolding, functional amyloid, and human disease
    Fabrizio Chiti
    Dipartimento di Scienze Biochimiche, Universita degli Studi di Firenze, I 50134 Firenze, Italy
    Annu Rev Biochem 75:333-66. 2006
    ....
  3. pmc Mechanisms underlying inflammation in neurodegeneration
    Christopher K Glass
    Department of Cellular and Molecular Medicine, University of California, San Diego, La Jolla, 92093, USA
    Cell 140:918-34. 2010
    Inflammation is associated with many neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, and multiple sclerosis...
  4. ncbi HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS
    Udai Bhan Pandey
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Nature 447:859-63. 2007
    A prominent feature of late-onset neurodegenerative diseases is accumulation of misfolded protein in vulnerable neurons...
  5. ncbi Microglia and inflammation-mediated neurodegeneration: multiple triggers with a common mechanism
    Michelle L Block
    Neuropharmacology Section, MD F1 01, National Institute of Environmental Health Sciences, P O Box 12233, Research Triangle Park, NC 27709, USA
    Prog Neurobiol 76:77-98. 2005
    Inflammation, a common denominator among the diverse list of neurodegenerative diseases, has recently been implicated as a critical mechanism responsible for the progressive nature of neurodegeneration...
  6. ncbi Microglia-mediated neurotoxicity: uncovering the molecular mechanisms
    Michelle L Block
    Neuropharmacology Section, National Institute of Environmental Health Sciences, Research Triangle Park, North Carolina 27709, USA
    Nat Rev Neurosci 8:57-69. 2007
    Mounting evidence indicates that microglial activation contributes to neuronal damage in neurodegenerative diseases. Recent studies show that in response to certain environmental toxins and endogenous proteins, microglia can enter an ..
  7. pmc Self-propagation of pathogenic protein aggregates in neurodegenerative diseases
    Mathias Jucker
    Department of Cellular Neurology, Hertie Institute for Clinical Brain Research, University of Tubingen, D 72076 Tubingen, Germany
    Nature 501:45-51. 2013
    ..As a unifying pathogenic principle, the prion paradigm suggests broadly relevant therapeutic directions for a large class of currently intractable diseases. ..
  8. ncbi Oxidative stress and neurodegeneration: where are we now?
    Barry Halliwell
    Department of Biochemistry, Yong Loo Lin School of Medicine, National University of Singapore, Singapore
    J Neurochem 97:1634-58. 2006
    ..systems to prevent 'ongoing' oxidative damage, let alone the extra oxidative damage imposed by the neurodegenerative diseases. Indeed, increased oxidative damage, mitochondrial dysfunction, accumulation of oxidized aggregated ..
  9. pmc Mitochondrial dynamics--fusion, fission, movement, and mitophagy--in neurodegenerative diseases
    Hsiuchen Chen
    Division of Biology and Howard Hughes Medical Institute, California Institute of Technology, Pasadena, CA 91125, USA
    Hum Mol Genet 18:R169-76. 2009
    ..Moreover, several major neurodegenerative diseases--including Parkinson's, Alzheimer's and Huntington's disease--involve disruption of mitochondrial ..
  10. pmc The seeds of neurodegeneration: prion-like spreading in ALS
    Magdalini Polymenidou
    Ludwig Institute for Cancer Research and Department of Cellular and Molecular Medicine, University of California at San Diego, 9500 Gilman Drive, La Jolla, CA 92093 0670, USA
    Cell 147:498-508. 2011
    Misfolded proteins accumulating in several neurodegenerative diseases (including Alzheimer, Parkinson, and Huntington diseases) can cause aggregation of their native counterparts through a mechanism similar to the infectious prion protein'..
  11. doi Regulation of autophagy by mTOR-dependent and mTOR-independent pathways: autophagy dysfunction in neurodegenerative diseases and therapeutic application of autophagy enhancers
    Sovan Sarkar
    Whitehead Institute for Biomedical Research, Massachusetts Institute of Technology, 9 Cambridge Center, Cambridge, MA 02142, U S A
    Biochem Soc Trans 41:1103-30. 2013
    ..Emerging evidence indicates that compromised autophagy contributes to the aetiology of various neurodegenerative diseases related to protein conformational disorders by causing the accumulation of mutant proteins and cellular ..
  12. doi Protein folding stress in neurodegenerative diseases: a glimpse into the ER
    Soledad Matus
    Center for Molecular Studies of Cell, Institute of Biomedical Sciences, Faculty of Medicine, University of Chile, Santiago, Chile
    Curr Opin Cell Biol 23:239-52. 2011
    Several neurodegenerative diseases share common neuropathology, primarily featuring the presence in the brain of abnormal protein inclusions containing specific misfolded proteins...
  13. ncbi Dopaminergic loss and inclusion body formation in alpha-synuclein mice: implications for neurodegenerative disorders
    E Masliah
    Department of Neurosciences, Department of Pathology, University of California San Diego, La Jolla, CA 92093 0624, USA
    Science 287:1265-9. 2000
    ..These results suggest that accumulation of wild-type alpha-synuclein may play a causal role in Parkinson's disease and related conditions...
  14. ncbi Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila
    J S Steffan
    Department of Psychiatry and Human Behavior, Gillespie 2121, University of California, Irvine, California 92697, USA
    Nature 413:739-43. 2001
    Proteins with expanded polyglutamine repeats cause Huntington's disease and other neurodegenerative diseases. Transcriptional dysregulation and loss of function of transcriptional co-activator proteins have been implicated in the ..
  15. ncbi Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases
    Michael T Lin
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, Room F 610, 525 East 68th Street, New York 10021, USA
    Nature 443:787-95. 2006
    Many lines of evidence suggest that mitochondria have a central role in ageing-related neurodegenerative diseases. Mitochondria are critical regulators of cell death, a key feature of neurodegeneration...
  16. ncbi Neuronal alpha-synucleinopathy with severe movement disorder in mice expressing A53T human alpha-synuclein
    Benoit I Giasson
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, 3600 Spruce Street, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Neuron 34:521-33. 2002
    ..These mice demonstrate that A53T alpha-synuclein leads to the formation of toxic filamentous alpha-synuclein neuronal inclusions that cause neurodegeneration...
  17. pmc Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging
    Richard I Morimoto
    Department of Biochemistry, Molecular Biology, and Cell Biology, Rice Institute for Biomedical Research, Northwestern University, Evanston, Illinois 60208, USA
    Genes Dev 22:1427-38. 2008
    ....
  18. doi Multiple sclerosis: an immune or neurodegenerative disorder?
    Bruce D Trapp
    Department of Neurosciences, Lerner Research Institute, Cleveland Clinic, Cleveland, Ohio 44195, USA
    Annu Rev Neurosci 31:247-69. 2008
    ....
  19. doi Microglia and neurodegeneration: the role of systemic inflammation
    Colm Cunningham
    School of Biochemistry and Immunology and Trinity College Institute of Neuroscience, Trinity College, Dublin, Ireland
    Glia 61:71-90. 2013
    ..the preclinical and clinical evidence supporting a significant role for systemic inflammation in chronic neurodegenerative diseases will be discussed...
  20. ncbi Mitochondrial fusion protects against neurodegeneration in the cerebellum
    Hsiuchen Chen
    Division of Biology, California Institute of Technology, Pasadena, CA 91125, USA
    Cell 130:548-62. 2007
    ....
  21. doi Alterations of the microRNA network cause neurodegenerative disease
    Sébastien S Hébert
    Center for Human Genetics, K U Leuven, Herestraat 49 bus 602, B 3000 Leuven, Belgium
    Trends Neurosci 32:199-206. 2009
    ..Thus, miRNAs are moving rapidly center stage as key regulators of neuronal development and function in addition to important contributors to neurodegenerative disorder...
  22. doi Role of pro-inflammatory cytokines released from microglia in neurodegenerative diseases
    Joshua A Smith
    Division of Neurology, Department of Neurosciences, Medical University of South Carolina, 96 Jonathan Lucas Street, Suite 309 CSB, P O Box 250606, Charleston, SC 29425, USA
    Brain Res Bull 87:10-20. 2012
    ..Taken together, recent findings suggest that microglial activation and pro-inflammatory cytokines merit interest as targets in the treatment of neurodegenerative disorders...
  23. doi Role of axonal transport in neurodegenerative diseases
    Kurt J De Vos
    MRC Center for Neurodegeneration Research, Institute of Psychiatry, King s College, London SE5 8AF, United Kingdom
    Annu Rev Neurosci 31:151-73. 2008
    Many major human neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis (ALS), display axonal pathologies including abnormal accumulations of proteins and organelles...
  24. pmc Direct transfer of alpha-synuclein from neuron to astroglia causes inflammatory responses in synucleinopathies
    He Jin Lee
    Department of Anatomy, School of Medicine, Konkuk University, Seoul 143 701, Korea
    J Biol Chem 285:9262-72. 2010
    ..This transmission step is thus an important mediator of pathogenic glial responses and could qualify as a new therapeutic target...
  25. ncbi Glutamine repeats and neurodegeneration
    H Y Zoghbi
    Howard Hughes Medical Institute, Baylor College of Medicine, Houston, Texas 77030, USA
    Annu Rev Neurosci 23:217-47. 2000
    A growing number of neurodegenerative diseases have been found to result from the expansion of an unstable trinucleotide repeat...
  26. pmc Autophagy in neurodegenerative disorders: pathogenic roles and therapeutic implications
    Rebecca Banerjee
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, 525 East 68 th Street, A 501, New York, NY 10065
    Trends Neurosci 33:541-9. 2010
    ..in mitotic cells, autophagy is now recognized as an arbiter of neuronal survival and death decisions in neurodegenerative diseases. Studies using postmortem human tissue, genetic and toxin-induced animal and cellular models indicate ..
  27. pmc Amyloid in neurodegenerative diseases: friend or foe?
    Katie J Wolfe
    Department of Cell and Developmental Biology, School of Medicine, University of North Carolina at Chapel Hill, 107 Mason Farm Road, Chapel Hill, NC 27514, United States
    Semin Cell Dev Biol 22:476-81. 2011
    ..Paradoxically, recent studies also suggest a protective action of chaperones in their promotion of the assembly of large, tightly packed, benign aggregates that sequester toxic protein species...
  28. doi Epigenetic regulation of gene expression in physiological and pathological brain processes
    Johannes Graff
    Picower Institute for Learning and Memory, Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology MIT, Cambridge, Massachusetts 02139, USA
    Physiol Rev 91:603-49. 2011
    ..Clearly, the brain contains an epigenetic "hotspot" with a unique potential to not only better understand its most complex functions, but also to treat its most vicious diseases...
  29. pmc Probing mechanisms that underlie human neurodegenerative diseases in Drosophila
    M Jaiswal
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas 77030, USA
    Annu Rev Genet 46:371-96. 2012
    ....
  30. ncbi Diseases of unstable repeat expansion: mechanisms and common principles
    Jennifer R Gatchel
    Department of Neuroscience, Medical Scientist Training Program, Baylor College of Medicine, 1 Baylor Plaza, Houston, Texas 77030, USA
    Nat Rev Genet 6:743-55. 2005
    ..In all these diseases, the context of the expanded repeat and the abundance, subcellular localization and interactions of the proteins and RNAs that are affected have key roles in disease-specific phenotypes...
  31. pmc Seeded aggregation and toxicity of {alpha}-synuclein and tau: cellular models of neurodegenerative diseases
    Takashi Nonaka
    Department of Molecular Neurobiology, Tokyo Institute of Psychiatry, Tokyo 156 8585, USA
    J Biol Chem 285:34885-98. 2010
    The deposition of amyloid-like filaments in the brain is the central event in the pathogenesis of neurodegenerative diseases. Here we report cellular models of intracytoplasmic inclusions of α-synuclein, generated by introducing ..
  32. ncbi Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice
    Taichi Hara
    Department of Bioregulation and Metabolism, Tokyo Metropolitan Institute of Medical Science, Tokyo 113 8613, Japan
    Nature 441:885-9. 2006
    ..Autophagy may have a protective role against the development of a number of neurodegenerative diseases. Here we report that loss of autophagy causes neurodegeneration even in the absence of any disease-..
  33. ncbi Role of free radicals in the neurodegenerative diseases: therapeutic implications for antioxidant treatment
    B Halliwell
    Department of Biochemistry, Faculty of Medicine, National University of Singapore, Singapore
    Drugs Aging 18:685-716. 2001
    ..Little is known about the impact of dietary antioxidants upon the development and progression of neurodegenerative diseases, especially Alzheimer's disease...
  34. doi Aberrant microRNA expression in the brains of neurodegenerative diseases: miR-29a decreased in Alzheimer disease brains targets neurone navigator 3
    M Shioya
    Department of Bioinformatics and Molecular Neuropathology, Meiji Pharmaceutical University, Kiyose, Tokyo 204 8588, Japan
    Neuropathol Appl Neurobiol 36:320-30. 2010
    ..To identify miRNAs closely associated with neurodegeneration, we performed miRNA expression profiling of brain tissues of various neurodegenerative diseases.
  35. ncbi Aggresomes, inclusion bodies and protein aggregation
    R R Kopito
    Dept of Biological Sciences, Stanford University, Stanford, CA 94305 5020, USA
    Trends Cell Biol 10:524-30. 2000
    ..This microtubule-dependent inclusion body is called an aggresome...
  36. pmc Dynamin-related protein 1 and mitochondrial fragmentation in neurodegenerative diseases
    P Hemachandra Reddy
    Neurogenetics Laboratory, Division of Neuroscience, Oregon National Primate Research Center, Oregon Health and Science University, 505 NW 185th Avenue, Beaverton, OR 97006, USA
    Brain Res Rev 67:103-18. 2011
    ..developments of abnormal mitochondrial dynamics, mitochondrial fragmentation, and neuronal damage in neurodegenerative diseases, including Alzheimer's, Parkinson's, Huntington's, and amyotrophic lateral sclerosis...
  37. pmc Oxidative genome damage and its repair: implications in aging and neurodegenerative diseases
    Muralidhar L Hegde
    Department of Biochemistry and Molecular Biology, University of Texas Medical Branch, Galveston, TX 77555 1079, USA
    Mech Ageing Dev 133:157-68. 2012
    ..While defects in BER/SSBR proteins have been linked to hereditary neurodegenerative diseases, our recent studies implicated transition metal-induced inhibition of NEIL family DGs in sporadic ..
  38. pmc Mitochondrial dysfunction in neurodegenerative diseases
    Ashu Johri
    Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, NY, USA
    J Pharmacol Exp Ther 342:619-30. 2012
    b>Neurodegenerative diseases are a large group of disabling disorders of the nervous system, characterized by the relative selective death of neuronal subtypes...
  39. pmc CAG expansion induces nucleolar stress in polyglutamine diseases
    Ho Tsoi
    Laboratory of Drosophila Research, School of Life Sciences, Faculty of Science, The Chinese University of Hong Kong, Shatin, NT, Hong Kong, China
    Proc Natl Acad Sci U S A 109:13428-33. 2012
    ..Our work provides in vivo evidence that expanded CAG RNAs trigger nucleolar stress and induce apoptosis via p53 and describes a polyQ pathogenic mechanism that involves the nucleolus...
  40. ncbi Tauopathy in Drosophila: neurodegeneration without neurofibrillary tangles
    C W Wittmann
    Department of Pathology, Division of Neuropathology, Brigham and Women s Hospital and Harvard Medical School, 221 Longwood Avenue, Room 514, Boston, MA 02115, USA
    Science 293:711-4. 2001
    ..This fly model may allow a genetic analysis of the cellular mechanisms underlying tau neurotoxicity...
  41. pmc MicroRNAs in neurodegenerative diseases and their therapeutic potential
    Eunsung Junn
    Center for Neurodegenerative and Neuroimmunologic Diseases, Department of Neurology, UMDNJ Robert Wood Johnson Medical School, Piscataway, NJ 08854, USA
    Pharmacol Ther 133:142-50. 2012
    ..Altered expression of certain miRNA molecules in the brains of patients with neurodegenerative diseases such as Alzheimer and Parkinson suggests that miRNAs could have a crucial regulatory role in these ..
  42. doi Minocycline and neurodegenerative diseases
    Hye Sun Kim
    Department of Pharmacology, Seoul National University, College of Medicine, Seoul, Republic of Korea
    Behav Brain Res 196:168-79. 2009
    ..tolerance and the excellent penetration into the brain, minocycline has been clinically tried for some neurodegenerative diseases such as stroke, multiple sclerosis, spinal cord injury, amyotropic lateral sclerosis, Hungtington's ..
  43. doi Autophagy deregulation in neurodegenerative diseases - recent advances and future perspectives
    Zelda H Cheung
    Division of Life Science, Molecular Neuroscience Center and State Key Laboratory of Molecular Neuroscience, The Hong Kong University of Science and Technology, Hong Kong, China
    J Neurochem 118:317-25. 2011
    ..Recently, the involvement of autophagy in the pathophysiology of neurodegenerative diseases has attracted considerable interest because autophagy deregulation has been linked to some of these ..
  44. ncbi Oxidative stress induced-neurodegenerative diseases: the need for antioxidants that penetrate the blood brain barrier
    Y Gilgun-Sherki
    Department of Neurology and Felsenstein Medical Research Center, Rabin Medical Center, The Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
    Neuropharmacology 40:959-75. 2001
    Oxidative stress (OS) has been implicated in the pathophysiology of many neurological, particularly neurodegenerative diseases. OS can cause cellular damage and subsequent cell death because the reactive oxygen species (ROS) oxidize vital ..
  45. pmc Ageing and neuronal vulnerability
    Mark P Mattson
    Laboratory of Neurosciences, National Institute on Aging Intramural Research Program, Baltimore, Maryland 21224 6825, USA
    Nat Rev Neurosci 7:278-94. 2006
    ....
  46. doi The propagation of prion-like protein inclusions in neurodegenerative diseases
    Michel Goedert
    MRC Laboratory of Molecular Biology, Hills Road, Cambridge, CB2 0QH, UK
    Trends Neurosci 33:317-25. 2010
    The most common neurodegenerative diseases, including Alzheimer's disease and Parkinson's disease, are characterized by the misfolding of a small number of proteins that assemble into ordered aggregates in affected brain cells...
  47. ncbi ESCRT-III dysfunction causes autophagosome accumulation and neurodegeneration
    Jin A Lee
    Gladstone Institute of Neurological Disease and Department of Neurology, University of California, San Francisco, California 94158, USA
    Curr Biol 17:1561-7. 2007
    ..indicate that ESCRT-III dysfunction is associated with the autophagy pathway, suggesting a novel neurodegeneration mechanism that may have important implications for understanding FTD and other age-dependent neurodegenerative diseases.
  48. pmc Why neurodegenerative diseases are progressive: uncontrolled inflammation drives disease progression
    Hui Ming Gao
    Neuropharmacology Section, Laboratory of Pharmacology, National Institute of Environmental Health Sciences National Institutes of Health, Research Triangle Park, NC 27709, USA
    Trends Immunol 29:357-65. 2008
    b>Neurodegenerative diseases are a group of chronic, progressive disorders characterized by the gradual loss of neurons in discrete areas of the central nervous system (CNS)...
  49. doi Rapamycin and mTOR-independent autophagy inducers ameliorate toxicity of polyglutamine-expanded huntingtin and related proteinopathies
    S Sarkar
    Department of Medical Genetics, University of Cambridge, Cambridge Institute for Medical Research, Addenbrooke s Hospital, Hills Road, Cambridge CB2 0XY, UK
    Cell Death Differ 16:46-56. 2009
    ..In this review, we describe various drugs and pathways inducing autophagy, which may be potential therapeutic approaches for HD and related conditions...
  50. pmc Genome-wide screen for modifiers of ataxin-3 neurodegeneration in Drosophila
    Julide Bilen
    Department of Biology, University of Pennsylvania, Philadelphia, Pennsylvania, USA
    PLoS Genet 3:1950-64. 2007
    ..SCA3) is among the most common dominantly inherited ataxias, and is one of nine devastating human neurodegenerative diseases caused by the expansion of a CAG repeat encoding glutamine within the gene...
  51. doi Brain-derived neurotrophic factor in neurodegenerative diseases
    Chiara Zuccato
    Department of Pharmacological Sciences and Center for Stem Cell Research, University of Milan, Via Balzaretti 9, Milan, Italy
    Nat Rev Neurol 5:311-22. 2009
    ..In this article, we will review the current knowledge about the involvement of BDNF in a number of neurodegenerative diseases, with particular emphasis on Huntington disease, and will provide the rationale for and discuss the ..
  52. ncbi Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone HSP70
    J M Warrick
    Department of Biology, University of Pennsylvania, Philadelphia, Pennsylvania, USA
    Nat Genet 23:425-8. 1999
    At least eight inherited human neurodegenerative diseases are caused by expansion of a polyglutamine domain within the respective proteins. This confers dominant toxicity on the proteins, leading to dysfunction and loss of neurons...
  53. ncbi Chemokine receptors in the central nervous system: role in brain inflammation and neurodegenerative diseases
    Laetitia Cartier
    Biology of Ageing Laboratory, Department of Geriatrics, Geneva University Hospitals, 2 Chemin du Petit Bel Air, CH 1225 Chene Bourg, Switzerland
    Brain Res Brain Res Rev 48:16-42. 2005
    ..Evidence is emerging that chemokine receptors are also involved in neuronal death and hence neurodegenerative diseases. Chemokines may induce neuronal death either indirectly (e.g...
  54. pmc Debris clearance by microglia: an essential link between degeneration and regeneration
    H Neumann
    Neural Regeneration, Institute of Reconstructive Neurobiology, University Bonn and Hertie Foundation, Bonn, Germany
    Brain 132:288-95. 2009
    ..Insufficient clearance by microglia, prevalent in several neurodegenerative diseases and declining with ageing, is associated with an inadequate regenerative response...
  55. pmc E46K human alpha-synuclein transgenic mice develop Lewy-like and tau pathology associated with age-dependent, detrimental motor impairment
    Kristel L Emmer
    Department of Pharmacology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 286:35104-18. 2011
    ..Collectively, these findings provide insights in the mechanisms of formation of these aberrant proteinaceous inclusions and support the notion that α-syn aggregates are involved in the pathogenesis of human diseases...
  56. ncbi Microglial activation and its implications in the brain diseases
    S Thameem Dheen
    Department of Anatomy, Yong Loo Lin School of Medicine, National University of Singapore, Blk MD10, 4 Medical Drive, Singapore 117597
    Curr Med Chem 14:1189-97. 2007
    ..CNS) is believed to play an important role in the pathway leading to neuronal cell death in a number of neurodegenerative diseases including Parkinson's disease, Alzheimer's disease, prion diseases, multiple sclerosis and HIV-dementia...
  57. pmc Prion-like transmission of protein aggregates in neurodegenerative diseases
    Patrik Brundin
    Patrik Brundin is at the Neuronal Survival Unit, Wallenberg Neuroscience Center, Lund University, BMC A10, 221 84 Lund, Sweden
    Nat Rev Mol Cell Biol 11:301-7. 2010
    b>Neurodegenerative diseases are commonly associated with the accumulation of intracellular or extracellular protein aggregates...
  58. ncbi Toxic proteins in neurodegenerative disease
    J Paul Taylor
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, Laboratory of Neurogenetics, National Institute on Aging, National Institutes of Health, Bethesda, MD 20892, USA
    Science 296:1991-5. 2002
    ..Increased understanding of the cellular mechanisms for disposal of abnormal proteins and of the effects of toxic protein accumulation on neuronal survival may allow the development of rational, effective treatment for these disorders...
  59. pmc Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease
    Maria Filimonenko
    Centre for Cancer Biomedicine, University of Oslo and Department of Biochemistry, The Norwegian Radium Hospital, Montebello, N 0310 Oslo, Norway
    J Cell Biol 179:485-500. 2007
    ..CHMP2B were recently associated with frontotemporal dementia and amyotrophic lateral sclerosis (ALS), neurodegenerative diseases characterized by abnormal ubiquitin-positive protein deposits in affected neurons...
  60. pmc Intercellular (mis)communication in neurodegenerative disease
    Gwenn A Garden
    Department of Neurology, University of Washington, Seattle, WA 98195, USA
    Neuron 73:886-901. 2012
    b>Neurodegenerative diseases have been intensively studied, but a comprehensive understanding of their pathogenesis remains elusive...
  61. pmc Mechanism of oxidative stress in neurodegeneration
    Sonia Gandhi
    Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK
    Oxid Med Cell Longev 2012:428010. 2012
    ..However, the promise of antioxidants as novel therapies for neurodegenerative diseases has not been borne out in clinical studies...
  62. pmc Dysregulation of cholesterol balance in the brain: contribution to neurodegenerative diseases
    Jean E Vance
    Group on the Molecular and Cell Biology of Lipids and Department of Medicine, University of Alberta, Edmonton, AB, T6G 2S2, Canada
    Dis Model Mech 5:746-55. 2012
    ..Some important considerations for treatment of neurodegenerative diseases are the impermeability of the blood-brain barrier to many therapeutic agents and difficulties in ..
  63. doi The benefits and limitations of animal models for translational research in neurodegenerative diseases
    Mathias Jucker
    Department of Cellular Neurology, Hertie Institute for Clinical Brain Research, University of Tubingen, Tubingen, Germany
    Nat Med 16:1210-4. 2010
    Age-related neurodegenerative diseases are largely limited to humans and rarely occur spontaneously in animals...
  64. pmc Expression of human FUS/TLS in yeast leads to protein aggregation and cytotoxicity, recapitulating key features of FUS proteinopathy
    Kazuo Fushimi
    Department of Neurology, Center for Genetic Medicine, Lurie Comprehensive Cancer Center, Northwestern University Feinberg School of Medicine, 303 E Superior, Chicago, IL 60611, USA
    Protein Cell 2:141-9. 2011
    ..FUS-positive neuropathology is reported in a range of neurodegenerative diseases, including ALS and fronto-temporal lobar degeneration with ubiquitin-positive pathology (FTLDU)...
  65. ncbi Oxidative stress and mitochondrial dysfunction in neurodegenerative diseases
    E Trushina
    Department of Molecular Pharmacology and Experimental Therapeutics, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
    Neuroscience 145:1233-48. 2007
    ..Emerging data from a number of neurodegenerative diseases suggest that there may be common features of toxicity that are related to oxidative damage...
  66. ncbi Review on the protective effects of PACAP in models of neurodegenerative diseases in vitro and in vivo
    D Reglodi
    Department of Anatomy, University of Pecs, Hungary
    Curr Pharm Des 17:962-72. 2011
    ..In vivo, PACAP shows neuroprotection in models of ischemic and traumatic brain injuries, and those of neurodegenerative diseases. The present review summarizes the findings on the neuroprotective potential of PACAP in models of ..
  67. doi Epigenetic programming of neurodegenerative diseases by an adverse environment
    Olena Babenko
    Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada
    Brain Res 1444:96-111. 2012
    ..The study of epigenetic states in neurodegenerative diseases presents an opportunity to gain new insights into risk factors and pathogenic mechanisms...
  68. pmc Neurodegenerative diseases: Lessons from genome-wide screens in small model organisms
    Tjakko J van Ham
    Department of Genetics, University Medical Centre Groningen and University of Groningen, Groningen, The Netherlands
    EMBO Mol Med 1:360-70. 2009
    Various age-related neurodegenerative diseases, including Parkinson's disease, polyglutamine expansion diseases and Alzheimer's disease, are associated with the accumulation of misfolded proteins in aggregates in the brain...
  69. ncbi Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract
    C L Wellington
    Centre for Molecular Medicine and Therapeutics and Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia V6T 1Z4, Canada
    J Biol Chem 273:9158-67. 1998
    The neurodegenerative diseases Huntington disease, dentatorubropallidoluysian atrophy, spinocerebellar atrophy type 3, and spinal bulbar muscular atrophy are caused by expansion of a polyglutamine tract within their respective gene ..
  70. pmc The BMP signaling pathway at the Drosophila neuromuscular junction and its links to neurodegenerative diseases
    Vafa Bayat
    Program in Developmental Biology, Baylor College of Medicine, Houston, TX, USA
    Curr Opin Neurobiol 21:182-8. 2011
    ..neuromuscular junction (NMJ) has recently provided new insights into the roles of various proteins in neurodegenerative diseases including Amyotrophic Lateral Sclerosis (ALS), Spinal Muscular Atrophy (SMA), Multiple Sclerosis (MS) ..
  71. ncbi ER stress and neurodegenerative diseases
    D Lindholm
    Department of Neuroscience, Unit of Neurobiology, Uppsala University, Biomedical Centre, Box 587, S 751 23 Uppsala, Sweden
    Cell Death Differ 13:385-92. 2006
    ..Here we will discuss the possible role of ER stress in neurodegenerative diseases, and highlight current knowledge in this field that may reveal novel insight into disease mechanisms and ..
  72. ncbi MicroRNA pathways modulate polyglutamine-induced neurodegeneration
    Julide Bilen
    Department of Biology, University of Pennsylvania, Philadelphia, PA 19104, USA
    Mol Cell 24:157-63. 2006
    Nine human neurodegenerative diseases are due to expansion of a CAG repeat- encoding glutamine within the open reading frame of the respective genes...
  73. doi Ubiquitin, the proteasome and protein degradation in neuronal function and dysfunction
    Hwan Ching Tai
    Division of Chemistry of Chemical Engineering, California Institute of Technology, Pasadena, California 91125, USA
    Nat Rev Neurosci 9:826-38. 2008
    ..Moreover, the discovery of ubiquitin-positive protein aggregates in a wide spectrum of neurodegenerative diseases underlines the importance and vulnerability of the degradative system in neurons...
  74. ncbi Neurodegenerative diseases and oxidative stress
    Kevin J Barnham
    Department of Pathology, The University of Melbourne, The Mental Health Research Institute of Victoria, Victoria 3010, Australia
    Nat Rev Drug Discov 3:205-14. 2004
    ..However, this same complexity provides a number of therapeutic targets, and different strategies, including novel metal-protein attenuating compounds, aimed at a variety of targets have shown promise in clinical studies...
  75. ncbi The roles of intracellular protein-degradation pathways in neurodegeneration
    David C Rubinsztein
    Department of Medical Genetics, Cambridge Institute for Medical Research, Addenbrooke s Hospital, Hills Road, Cambridge CB2 2XY
    Nature 443:780-6. 2006
    Many late-onset neurodegenerative diseases, including Parkinson's disease and Huntington's disease, are associated with the formation of intracellular aggregates by toxic proteins...
  76. pmc Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration
    Edward B Lee
    Translational Neuropathology Research Laboratory, Division of Neuropathology, Department of Pathology and Laboratory Medicine, University of Pennsylvania, 605B Stellar Chance Laboratories, 422 Curie Boulevard, Philadelphia, Pennsylvania 19104, USA
    Nat Rev Neurosci 13:38-50. 2011
    ..In addition, the distinct possibility of pleotropic or combined effects - in which gains of toxic properties and losses of normal TDP43 functions act together - needs to be considered...
  77. ncbi Initiation and synergistic fibrillization of tau and alpha-synuclein
    Benoit I Giasson
    Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine
    Science 300:636-40. 2003
    ..and tau polymerize into amyloid fibrils and form intraneuronal filamentous inclusions characteristic of neurodegenerative diseases. We demonstrate that alpha-syn induces fibrillization of tau and that coincubation of tau and alpha-syn ..
  78. pmc Neuroinflammation and oxidation/nitration of alpha-synuclein linked to dopaminergic neurodegeneration
    Hui Ming Gao
    Department of Pathology and Laboratory Medicine, Center for Neurodegenerative Disease Research, Institute on Aging, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Neurosci 28:7687-98. 2008
    ..This study advances understanding of the role of neuroinflammation and abnormal SYN in the pathogenesis of PD and opens new avenues for the discovery of more effective therapies for PD...
  79. pmc Drosophila models of neurodegenerative diseases
    Bingwei Lu
    Department of Pathology, Stanford University School of Medicine, Stanford, CA 94305, USA
    Annu Rev Pathol 4:315-42. 2009
    b>Neurodegenerative diseases are progressive disorders of the nervous system that affect specific cellular populations in the central and peripheral nervous systems...
  80. ncbi Innate immunity: the missing link in neuroprotection and neurodegeneration?
    Minh Dang Nguyen
    Centre for Research in Neurosciences, McGill University, The Montreal General Hospital Research Institute, Montreal, Quebec, H3G 1A4, Canada
    Nat Rev Neurosci 3:216-27. 2002
    ..Paradoxically, this response also protects neurons by favouring remyelination and trophic support afforded by glial cells...
  81. ncbi Glutathione pathways in the brain
    Ralf Dringen
    Physiologisch chemisches Institut der Universität Tübingen, Hoppe Seyler Str 4, D 72076 Tubingen, Germany
    Biol Chem 384:505-16. 2003
    ..In addition, implications of disturbed GSH pathways in brain for neurodegenerative diseases will be discussed.
  82. doi Polyglutamine diseases: where does toxicity come from? what is toxicity? where are we going?
    Toshiaki Takahashi
    School of Health Sciences, Faculty of Medicine, Niigata University, 1 757 Asahimachi, Niigata 951 8122, Japan
    J Mol Cell Biol 2:180-91. 2010
    ..We review the current understanding of the toxic structure and dysfunction by expanded polyglutamine proteins and suggest directions for future studies of polyglutamine diseases...
  83. pmc The genetic epidemiology of neurodegenerative disease
    Lars Bertram
    Genetics and Aging Research Unit, MassGeneral Institute for Neurodegenerative Diseases, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Charlestown, Massachusetts 02129, USA
    J Clin Invest 115:1449-57. 2005
    ..In this review, we discuss the current status of genetic epidemiology of the most common neurodegenerative diseases: Alzheimer disease, Parkinson disease, Lewy body dementia, frontotemporal dementia, amyotrophic lateral ..
  84. ncbi Unfolding the role of protein misfolding in neurodegenerative diseases
    Claudio Soto
    Serono International, 15 Chemin des Mines, Geneva, Switzerland
    Nat Rev Neurosci 4:49-60. 2003
  85. ncbi Protein aggregation and neurodegenerative disease
    Christopher A Ross
    Division of Neurobiology, Department of Psychiatry, Johns Hopkins University School of Medicine, Ross Research Building, Room 618, 720 Rutland Avenue, Baltimore, Maryland 21205, USA
    Nat Med 10:S10-7. 2004
    b>Neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), amyotrophic lateral sclerosis (ALS) and prion diseases are increasingly being realized to have common cellular and molecular ..
  86. pmc Inflammation in neurodegenerative diseases
    Sandra Amor
    Department of Pathology, VU University Medical Centre De Boelelaan, Amsterdam, The Netherlands
    Immunology 129:154-69. 2010
    ..of the interaction between the immune and nervous systems will be crucial to either target pathogenic responses, or augment the beneficial effects of immune responses as a strategy to intervene in chronic neurodegenerative diseases.
  87. pmc Cell-to-cell transmission of pathogenic proteins in neurodegenerative diseases
    Jing L Guo
    Department of Pathology and Laboratory Medicine, Institute on Aging and Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, USA
    Nat Med 20:130-8. 2014
    A common feature of many neurodegenerative diseases is the deposition of β-sheet-rich amyloid aggregates formed by proteins specific to these diseases...
  88. doi Review: Role of developmental inflammation and blood-brain barrier dysfunction in neurodevelopmental and neurodegenerative diseases
    H B Stolp
    Department of Pharmacology, University of Melbourne, Parkville, Victoria, Australia
    Neuropathol Appl Neurobiol 35:132-46. 2009
    ..blood-brain barrier function (integrity), which is universally important in both neurodevelopmental and neurodegenerative diseases, is proposed...
  89. ncbi Resveratrol and neurodegenerative diseases: activation of SIRT1 as the potential pathway towards neuroprotection
    Merce Pallas
    Facultat de Farmacia, Institut de Biomedicina IBUB, Centro de Investigación de Biomedicina en Red de Enfermedades Neurodegenerativas, Universitat de Barcelona, Nucli Universitari de Pedralbes, Barcelona, Spain
    Curr Neurovasc Res 6:70-81. 2009
    ..In addition, we discuss the possibility of using resveratrol in the treatment of neurodegenerative diseases.
  90. pmc Viral vectors for neurotrophic factor delivery: a gene therapy approach for neurodegenerative diseases of the CNS
    Seung T Lim
    Department of Neuroscience, Georgetown University Medical Center, Washington, DC 20057, United States
    Pharmacol Res 61:14-26. 2010
    ..In this review, we discuss viral vector-mediated gene transfer of NTFs to treat neurodegenerative diseases of the central nervous system.
  91. pmc Retrograde axonal transport: pathways to cell death?
    Eran Perlson
    Department of Physiology, University of Pennsylvania School of Medicine, Philadelphia, PA 19067, USA
    Trends Neurosci 33:335-44. 2010
    ..Defects in transport are sufficient to induce neurodegeneration, but recent progress suggests that changes in retrograde signaling pathways correlate with rapidly progressive neuronal cell death...
  92. ncbi Modifiers and mechanisms of multi-system polyglutamine neurodegenerative disorders: lessons from fly models
    Moushami Mallik
    Cytogenetics Laboratory, Department of Zoology, Banaras Hindu University, Varanasi 221 005, India
    J Genet 89:497-526. 2010
    ..Such network effects pose a major challenge for therapeutic applications...
  93. doi Multiple sclerosis as a neurodegenerative disease: pathology, mechanisms and therapeutic implications
    Christine Stadelmann
    Institute of Neuropathology, University Medical Centre, Gottingen, Germany
    Curr Opin Neurol 24:224-9. 2011
    ..This has led to the hypothesis that disease mechanisms independent of an adaptive immune response contribute to disease progression and closely resemble neurodegeneration...
  94. pmc Mitochondria as a therapeutic target for aging and neurodegenerative diseases
    P H Reddy
    Neurogenetics Laboratory, Division of Neuroscience, Oregon National Primate Research Center, Oregon Health and Science University, Beaverton, OR 97006, USA
    Curr Alzheimer Res 8:393-409. 2011
    ..Extensive evidence from animal models, postmortem brain studies of and clinical studies of aging and neurodegenerative diseases suggests that mitochondrial function is defective in aging and neurodegenerative diseases, such as ..
  95. doi The role of mitochondria in neurodegenerative diseases
    Massimiliano Filosto
    Clinical Neurology, Section for Neuromuscular Diseases and Neuropathies, University Hospital Spedali Civili, Pz le Spedali Civili 1, 25100, Brescia, Italy
    J Neurol 258:1763-74. 2011
    ..Mitochondrial abnormalities have been documented in neurodegenerative diseases, including Alzheimer's, Parkinson's, and Huntington's diseases, and amyotrophic lateral sclerosis...
  96. doi Double-stranded RNA is pathogenic in Drosophila models of expanded repeat neurodegenerative diseases
    Kynan T Lawlor
    ARC Special Research Centre for the Molecular Genetics of Development and Discipline of Genetics, School of Molecular and Biomedical Sciences, The University of Adelaide, Adelaide, SA 5005, Australia
    Hum Mol Genet 20:3757-68. 2011
    The pathogenic agent responsible for the expanded repeat diseases, a group of neurodegenerative diseases that includes Huntington's disease is not yet fully understood...
  97. doi Modeling human neurodegenerative diseases in transgenic systems
    Miguel A Gama Sosa
    Research and Development Service, James J Peters Department of Veterans Affairs Medical Center, Bronx, NY 10468, USA
    Hum Genet 131:535-63. 2012
    Transgenic systems are widely used to study the cellular and molecular basis of human neurodegenerative diseases. A wide variety of model organisms have been utilized, including bacteria (Escherichia coli), plants (Arabidopsis thaliana), ..
  98. ncbi Mitochondrial dysfunction in neurodegenerative diseases
    M F Beal
    Neurology Service WRN 408, Massachusetts General Hospital and Harvard Medical School, 32 Fruit Street, Boston, MA 02114, USA
    Biochim Biophys Acta 1366:211-23. 1998
    A potential pivotal role for mitochondrial dysfunction in neurodegenerative diseases is gaining increasing acceptance...
  99. pmc Role for neuronal insulin resistance in neurodegenerative diseases
    Markus Schubert
    Institute for Genetics, University of Cologne, D 50931 Cologne, Germany
    Proc Natl Acad Sci U S A 101:3100-5. 2004
    Impairment of insulin signaling in the brain has been linked to neurodegenerative diseases. To test the hypothesis that neuronal insulin resistance contributes to defects in neuronal function, we have performed a detailed analysis of ..
  100. ncbi Mitochondrial dysfunction, oxidative stress, regulation of exocytosis and their relevance to neurodegenerative diseases
    Damien J Keating
    Department of Human Physiology and Centre for Neuroscience, Flinders University, Adelaide, Australia
    J Neurochem 104:298-305. 2008
    A common feature in the early stages of many neurodegenerative diseases lies in mitochondrial dysfunction, oxidative stress, and reduced levels of synaptic transmission...
  101. ncbi The many faces of corticobasal degeneration
    Pettarusp M Wadia
    Movement Disorders Centre, Toronto Western Hospital, University of Toronto, Canada
    Parkinsonism Relat Disord 13:S336-40. 2007
    ..CBS is a sensitive clinical phenotype for tau pathology. Patients with CBS could potentially be candidates for therapies aiming to modify tau biochemistry...

Research Grants81

  1. NIH Director's Pioneer Award
    Junying Yuan; Fiscal Year: 2009
    ..No Abstract provided ..
  2. Enhanced iron removal with peptide linked chelators targeted to ferritin
    Elizabeth C Theil; Fiscal Year: 2010
    ..for clinical trials to improve the treatment of Sickle Cell Disease and Thalassemia and possibly for neurodegenerative diseases currently being considered for iron chelation therapy...
  3. Gamma-Synuclein-Mediated Regulation of Norepinephrine Transporter
    Anita Sidhu; Fiscal Year: 2010
    ..NE] neurotransmission in the human brain is linked to mood disorders, depression, drug addiction and neurodegenerative diseases. -synuclein [-Syn], a member of the synuclein family of proteins, is expressed in monoaminergic neurons, ..
  4. Sphingolipid Metabolism in Drosophila Development
    Julie D Saba; Fiscal Year: 2013
    ..Excessive autophagy underlies many human disease states including neurodegenerative diseases, cardiomyopathies, cancer, programmed cell death, and bacterial and viral infections...
  5. Samuel D Crish; Fiscal Year: 2016
    ..This proposal is designed with the goal of identifying new therapeutic targets for this disease as well as identifying the potential role that function-restoring therapies could play in glaucoma and other neurodegenerative diseases.
  6. Inderjit Singh; Fiscal Year: 2014
    ..of new therapeutic targets for induction of myelin repair for improved treatment and management of neurodegenerative diseases such as MS...
  7. DHAN V KALVAKOLANU; Fiscal Year: 2014
    ..Studies proposed in this application will investigate the regulation of an anti-metastatic gene, DAPK1, which is critical for tumor suppression and autophagic responses. ..
  8. Monitoring cellular redox signaling and oxidant stress in vivo
    Paul T Schumacker; Fiscal Year: 2010
    ..cellular dysfunction associated with ischemia- reperfusion injury, vascular disease, stroke, diabetes, neurodegenerative diseases, liver disease, renal disease, inflammation, cancer, and other disorders...
  9. Enzymatic Regulation of S-Glutathionylation : Role in Apoptosis and Disease
    John J Mieyal; Fiscal Year: 2012
    ..especially glutaredoxin and thioredoxin, are critical to mitigating the effects of oxidative stress in neurodegenerative diseases, as well as in cardiovascular disease, diabetes and aging, all of which are prevalent among veterans...
  10. MARK L DELL'ACQUA; Fiscal Year: 2016
    ..for understanding how excessive glutamate receptor activation leads to excitotoxic neuronal death in neurodegenerative diseases, brain injury and stroke...
  11. Drug Interactions at the Human Blood-Brain Barrier
    Jashvant D Unadkat; Fiscal Year: 2010
    ..Fluid Biomarkers for Early Diagnosis and Progression of Aging-related Diseases and Conditions including Neurodegenerative Diseases. The blood brain barrier (BBB) is a significant barrier to delivery of drugs to the central nervous ..
  12. Cellular and Animal Models of FUS Mutations in ALS
    Eric J Huang; Fiscal Year: 2013
    ..Since synaptic degeneration plays a central role in neurodegenerative diseases, we believe that the establishment of these models will contribute to understanding of the mutant FUS ..
  13. Ai Ling Lin; Fiscal Year: 2016
    ..Translational neuroimaging is an emerging field with extraordinary promise. My ambition is to become pioneer in this emerging discipline. ..
  14. Identification of Compounds to treat Charcot-Marie-Tooth type 2E neuropathy
    RONALD K LIEM; Fiscal Year: 2010
    ..in the neuronal cell bodies and axons are seen in patients with mutations in NEFL, as well as in other neurodegenerative diseases, such as amyotrophic lateral sclerosis...
  15. Efficacy of GABAA a5 receptor inverse agonists in learning impaired rats
    EARL MICHAEL GIBBS; Fiscal Year: 2010
    ..Patients with learning disorders and those suffering from complications due to neurodegenerative diseases will greatly benefit as PWZ-029 moves another step closer to the clinic.
  16. Regulation of Astrocyte TIMP-1 in HIV-Associated Dementia
    JEREL ADAM FIELDS; Fiscal Year: 2011
    ..Our studies will provide a better understanding of the specific mechanistic contributions of activated astrocytes to HIV-1-neuropathogensis and neuroinflammation. ..
  17. IRON REGULATION OF GENE EXPRESSION
    ELIZABETH ANN LEIBOLD; Fiscal Year: 2013
    ..Iron dysregulation leads to hematological, metabolic and neurodegenerative diseases, and increases the risk of cancer...
  18. Moosa Mohammadi; Fiscal Year: 2016
    ..Since substrate recruitment and phosphorylation by RTKs is a general event in RTK signaling, the mechanistic insights gained under Aim IV will also be directly applicable to the entire RTK superfamily. ..
  19. 14th International Workshop on Ataxia-Telangiectasia and ATM
    Richard A Gatti; Fiscal Year: 2010
    ..an international group of basic and clinical researchers working on various aspects of A-T and related neurodegenerative diseases, as well as on the role of ATM in cancer, the DNA damage response, and in viral infections that involve ..
  20. Molecular Pathological Mechanism & Potential Therapeutics of Lafora Disease
    YAN NONE LIU; Fiscal Year: 2010
    ..Two common pathological causes for developing neurodegenerative diseases are diverse abnormal aggregates formed inside or outside of affected neuronal cells and endoplasmic ..
  21. Chromatin Modifications and Vulnerability to Glutamate Toxicity
    ALEXEI D KONDRATYEV; Fiscal Year: 2010
    ..neurological insults such as ischemia and seizures, as well as contributes to neuronal death in neurodegenerative diseases (Alzheimer's, Parkinson's, etc.)...
  22. Role of Dendritic Ca2+ Transients in Respiratory Rhythm Generation
    Jack L Feldman; Fiscal Year: 2010
    ..Various neurodegenerative diseases, such as Parkinson's disease, multiple systems atrophy and amyotrophic lateral sclerosis, are ..
  23. Fall risk assessment and prevention in elderly with diabetes using wearable techn
    Bor Rong Chen; Fiscal Year: 2012
    ..The proposed technology has a very wide range of applications in elderly care, stroke, neurodegenerative diseases, traumatic brain injury (TBI), and in treating balance disorders and dizziness...
  24. RNA interference therapy for Huntington's disease: studies in non-human primates
    Beverly L Davidson; Fiscal Year: 2010
    ..One, is long-term application of therapeutic RNAi to primate brain feasible for chronic neurodegenerative diseases like HD? Second, is the primate brain tolerant of knock down of both normal and mutant HTT alleles? To ..
  25. Testing the efficacy of PGC-1alpha as a therapeutic target for Parkinson's diseas
    HANS BUELER; Fiscal Year: 2010
    ..these results suggest that PGC-1a is a prime candidate therapeutic protein for the treatment of neurodegenerative diseases that are caused by mitochondrial dysfunction and oxidative stress, such as PD and amyotrophic lateral ..
  26. Jianxiong Jiang; Fiscal Year: 2014
    ....
  27. MOUSE MODEL FOR DISEASES OF PROTEIN MISFOLDING
    P Michael Conn; Fiscal Year: 2012
    ..hypogonadism, nephrogenic diabetes insipidus, retinitis pigmentosa, hypercholesterolemia, cataracts, neurodegenerative diseases (Huntington's, Alzheimer's and Parkinson's), cancers and digestive disorders...
  28. Scott R Barnum; Fiscal Year: 2014
    ..motor neuron loss in ALS, due in large part to the recognition of immune-mediated contributions to other neurodegenerative diseases, including multiple sclerosis, Alzheimer's and Parkinson's disease...
  29. Kip E Guja; Fiscal Year: 2014
    ..Clear associations exist between deficiencies in mitochondrial function and neurodegenerative diseases such as Alzheimer's and Parkinson's disease, as well as diabetes and cancer...
  30. Oxidative stress and neurogenetic networks in Drosophila
    Robert R H Anholt; Fiscal Year: 2010
    ..stress resulting from chemical exposure has been implicated as a contributing risk factor for a range of neurodegenerative diseases. However, it has been challenging to dissect genetic and environmental contributions to phenotypes that ..
  31. FASEB SRC on Mitochondrial Assembly &Dynamics in Health, Disease &Aging
    Antoni Barrientos; Fiscal Year: 2011
    ..In addition, the role of mitochondria in general neurodegenerative diseases such as Parkinson's and during the aging process will be discussed in sessions highlighting ..
  32. GLUTAMATE TRANSPORTERS IN THE CNS
    MICHAEL BYRNE ROBINSON; Fiscal Year: 2011
    ..In addition, several acute and chronic neurodegenerative diseases (or animal models of these diseases) are accompanied by decreased expression of GLT-1/EAAT2...
  33. A new ultra-low field in-vivo EPR technology for biomedical applications
    Inseob Hahn; Fiscal Year: 2011
    ....
  34. A new ultra-low field in-vivo EPR technology for biomedical applications
    Inseob Hahn; Fiscal Year: 2012
    ....
  35. Sulie L Chang; Fiscal Year: 2014
    ..Increased inflammation in the brain has been correlated with neurodegenerative diseases and behavioral disorders, such as HIV-1 induced neurodegeneration (HAND) and HIV-1 induced dementia (HAD)..
  36. Innate Immune Mechanisms of Motor Neuron Injury
    Timothy Vartanian; Fiscal Year: 2010
    DESCRIPTION (provided by applicant): Health Relatedness: A central question in neurodegenerative diseases is how specific populations of neurons undergo cell death...
  37. Jian Yang; Fiscal Year: 2015
    ..As intracellular calcium signaling is severely altered in aging neurons and age-related neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, we will investigate whether mid-channel ..
  38. Multiple Mechanisms of Nasal Chemoreception
    Diego Restrepo; Fiscal Year: 2012
    ..Reference List Doty,R.L. (1991). Olfactory dysfunction in neurodegenerative diseases. In Smell and taste in health and disease, T.V. Getchell, R.L. Doty, L.M. Bartoshuk, and J.B. Snow, Jr...
  39. Microfluidic-microtiter interface for rapid large-scale screenings of C. elegans
    Adela Ben Yakar; Fiscal Year: 2010
    ..the medical handling of traumatic nerve injuries and will also offer valuable insights regarding many neurodegenerative diseases. The use of large vertebrate animal models for gene identification requires complex assays and ..
  40. In vivo analysis of the mechanisms of axon transport.
    Catherine M Drerup; Fiscal Year: 2011
    ..Defects in this process are associated with numerous developmental and neurodegenerative diseases such as Spinal Muscular Atrophy, Alzheimer's Disease, and Amyotrophic Lateral Sclerosis...
  41. Energy Balance During Ketosis in Rat Brain
    JOSEPH CHARLES LAMANNA; Fiscal Year: 2010
    ..Based on animal studies, ketosis has been suggested to possess neuroprotective properties against neurodegenerative diseases such as Alzheimer's, Parkinson's and recovery from focal stroke...
  42. Ralph A Nixon; Fiscal Year: 2014
    ..In several major neurodegenerative diseases, including a form of fronto-temporal dementia, abnormal accumulations of NF are a neuropathologic ..
  43. Klotho regulation and aging
    Gwendalyn D King; Fiscal Year: 2010
    ..The aging suppressor protein, Klotho is decreased in the brain with age and may be a target for therapeutic development against neurodegenerative disorders. ..
  44. RNA polymerase II Elongation Complex: Structure and Function
    Daniel Reines; Fiscal Year: 2013
    ..The human ortholog of SEN1, SETX, is of biomedical importance since its dysfunction results in the neurodegenerative diseases Ataxia and Ocular Apraxia (AOA) type 2 and Amyotrophic Lateral Sclerosis (ALS) type 4...
  45. Regulation of the Expression of G[alpha]i2 by Reactive Oxygen Species
    Ifeanyi J Arinze; Fiscal Year: 2012
    ....
  46. Samay Jain; Fiscal Year: 2015
    ..with resources for recruitment and retention of geriatric participants;the Pittsburgh Institute for Neurodegenerative diseases;and the Comprehensive Movement Disorders Center...
  47. Presynaptic Mechanisms Regulating the Dopamine Transporter
    JAMES A HARDAWAY; Fiscal Year: 2013
    ..C. elegans express many of the same genes within their DA neurons as higher order organisms, so we believe our screen may reveal conserved genes that regulate DA levels in humans. ..
  48. Molecular Regulation of Alpha-Synuclein Misfolding and Toxicity in Two Yeast Mode
    SHUBHIK DEBBURMAN; Fiscal Year: 2009
    ..Director (Last, first, middle): DebBurman, Shubhik, Kumar PROJECT RELEVANCE The growing list of neurodegenerative diseases, including Parkinson's disease, represents a burgeoning public health concern in the United States...
  49. AAA ATPases linking mitochondria with microtubule processing in flies and yeast
    Karen G Hales; Fiscal Year: 2012
    ..PUBLIC HEALTH RELEVANCE: Defects of mitochondrial shaping and movement underlie many neurodegenerative diseases and may be associated with premature aging as well as infertility...
  50. Analysis of FIP200 in signal transduction
    Jun Lin Guan; Fiscal Year: 2012
    ..and cellular mechanisms by which FIP200 regulation of signaling pathways and cellular processes in the neurodegenerative diseases. Based on our previous and preliminary studies, we propose to 1)...
  51. ALS therapies and genomics for mutant TDP-43 and TLS/FUS
    Don W Cleveland; Fiscal Year: 2010
    ..cytoplasmic inclusions are found in the vast majority of ALS sporadic patients and in several other neurodegenerative diseases including Frontal Temporal Dementia (FTD), errors in TDP-43 may be central to understanding disease ..
  52. ERNESTO ROQUE BONGARZONE; Fiscal Year: 2016
    ....
  53. Amit Berson; Fiscal Year: 2015
    ..the roles that such modifications play in maintenance of the nervous system and their contribution to neurodegenerative diseases remain largely unknown...
  54. Effects of nerve demyelination on auditory synaptic functions
    Jun Hee Kim; Fiscal Year: 2012
    ..in auditory nerve fibers, as well as this proposal has a broad relevance for the entire field of neurodegenerative diseases. The proposed study will lay the foundations for the development of novel therapeutic strategies for ..
  55. Astrocytes Play a Critical Role in the Pathology of EAE
    JOHN ROLAND BETHEA; Fiscal Year: 2013
    ..In all these processes, in MS as well as in many other neurodegenerative diseases, astrocytes have been demonstrated to play an active role...
  56. Copper's Role in Brain LRP-mediated Abeta Efflux and Aging
    Rashid Deane; Fiscal Year: 2010
    ..These studies may provide new therapeutic insights on how to lower brain A[unreadable] by controlling its CNS barriers transport in Cu-potentiated neurodegenerative disorders. ..
  57. Barry M Willardson; Fiscal Year: 2016
    ..b>Neurodegenerative diseases such as Alzheimer's, Parkinson's, Huntington's and Lou Gehrig's disease (amyotrophic ..
  58. Testing Compounds that Increase SMN levels for Efficacy in Mouse Models of SMA
    Elliot J Androphy; Fiscal Year: 2010
    ..of three laboratories with expertise in SMA molecular biology, development of novel therapies for neurodegenerative diseases, and performance of drug trials in murine models of SMA...
  59. Neuroimmune modulation across the BBB: Regulation of ILS trafficking by TNF
    Weihong Pan; Fiscal Year: 2012
    ..Their interactions with the blood-brain barrier largely determine the final outcome of these blood-borne cytokines. ..
  60. International Conf on Alzheimer Disease and Related Disorders in the Middle East
    Changiz Geula; Fiscal Year: 2013
    ..The organizers have successfully convened five editions of this conference in the past, and based on past experience expect an even more successful sixth conference. ..
  61. Mechanisms of Ubiquitin Trafficking in Neurons
    Michael C Wooten; Fiscal Year: 2013
    ..stress, ubiquitination defects, and mitochondrial dysfunction are well recognized markers of various neurodegenerative diseases. This proposal is designed to elucidate the mechanism whereby the signaling scaffold, p62, regulates ..
  62. Christopher E Turner; Fiscal Year: 2016
    ....
  63. Androgen Modulation of Neurodegeneration in Dopamine Neurons
    Rebecca L Cunningham; Fiscal Year: 2010
    ..Nopage numbering necessary. Not to exceed 3 publications;2 for predoctoral candidates.) E3 Check if Appendix is included PHS 416-1 (Rev. 10/05) Page 4 Form Page 4 ..
  64. Characterization of de novo generation of infectious prions
    Joaquin Castilla; Fiscal Year: 2009
    ..This find opens enormous possibilities for understanding the prion diseases and for designing new therapies inconceivable until now. ..
  65. Non-Invasive Quantitation of Cellular and Biochemical Changes in Hereditary Ataxi
    GULIN OZ; Fiscal Year: 2009
    ..applicant): Successful application of potential neuroprotective agents will depend on early detection of neurodegenerative diseases. Biochemical and cellular processes that trigger and accompany the neuronal loss in these diseases are ..
  66. SIRT1 Overexpression in Cellular Mitochondrial Metabolism and Function
    James Russell; Fiscal Year: 2009
    ..by applicant): Oxidative stress and mitochondrial dysfunction have been associated with a wide range of neurodegenerative diseases and metabolic disorders such as diabetes...
  67. Histone Deacetylases and reversible acetylation in signaling and disease
    JOHN DENU; Fiscal Year: 2009
    ..Use of funds for this purpose will provide an excellent training opportunity for early-career scientists who will present their work and learn about the latest developments in this burgeoning field. ..
  68. Molecular mechanisms of oxidative stress resistance in an animal model of aging w
    Sarah Milton; Fiscal Year: 2009
    ..as key factors in aging and senescence, and ROS damage is thought to play a role in a number of neurodegenerative diseases including Alzheimer's, Parkinson's Disease, and stroke (ischemia/reperfusion)...
  69. Indicators of early neurodegeneration: gene-toxin interactions at the syanpse
    Felix E Schweizer; Fiscal Year: 2010
    ..risk factors as well as genetic predisposition underlie the development and progression of most neurodegenerative diseases. Although neuronal death characterizes the clinical stages of many of these diseases, dysfunction in the ..
  70. Celestrols for Treatment of Neurodegenerative Diseases
    Richard Silverman; Fiscal Year: 2007
    ..molecular chaperones should lead to the development of novel therapies beneficial to the prevention of neurodegenerative diseases. The rationale for this proposal is based on results obtained by our laboratory and others who ..
  71. Broadly Neuroprotective Drug for TBI
    Fernando Gomez Pinilla; Fiscal Year: 2010
    ..the precursor of CNB-001, has shown excellent efficacy in counteracting dysfunction in several models of neurodegenerative diseases such as Alzheimer's, cerebral ischemia, promoting neurogenesis in the adult hippocampus, and in our ..
  72. GLYCOSPHINGOLIPIDS IN MURINE NEURODEGENERATIVE DISEASES
    THOMAS SEYFRIED; Fiscal Year: 2009
    ..The gangliosidoses are a group of incurable neurodegenerative diseases involving storage of either ganglioside GM1 or GM2 in lysosomes...
  73. METABOTROPIC GLUTAMATE RECEPTORS IN NEURODEGENERATION
    Anne Young; Fiscal Year: 2009
    Human neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS) are characterized by adult onset, progressive neurologic ..
  74. Developmental Pesticide Exposure: The Parkinson's Disease Phenotype
    Mona Thiruchelvam; Fiscal Year: 2010
    ..Moreover, these findings will have implications for the derivation of biomarkers and therapeutic strategies for PD. PHS 398/2590 (Rev. 09/04, Reissued 4/2006) Page Continuation Format Page ..
  75. Neurodegeneration in Aging Retarded Mice
    Roger Albin; Fiscal Year: 2009
    ..Some unknown features of aging make the human brain susceptible to these degenerative disorders. We propose to develop new mouse models that will allow analysis of the interaction of normal aging and neurodegeneration. ..
  76. CHOLESTEROL AND NEURODEGENERATION
    Shutish Patel; Fiscal Year: 2001
    ....
  77. Oxidative modification of brain proteins in pesticide intoxication
    PIER MASTROBERARDINO; Fiscal Year: 2009
    ..The candidate is a postdoctoral fellow in the Pittsburgh Institute for Neurodegenerative Diseases (PIND), University of Pittsburgh, under the supervision of Dr. Tim Greenamyre. He received his Ph.D...