J Paul Taylor

Summary

Affiliation: National Institutes of Health
Country: USA

Publications

  1. ncbi request reprint 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
  2. ncbi request reprint Altered acetylation in polyglutamine disease: an opportunity for therapeutic intervention?
    J Paul Taylor
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, Building 10, Room 3B 14, Bethesda, MD 20892 1250, USA
    Trends Mol Med 8:195-7. 2002
  3. ncbi request reprint Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein
    J Paul Taylor
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, Building 10, Room 3B 14, Bethesda, MD 20892 1250, USA
    Hum Mol Genet 12:749-57. 2003
  4. pmc Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy
    Isabella Palazzolo
    Neurogenetics Branch, NINDS, NIH, Bethesda, MD 20892, USA
    Neuron 63:316-28. 2009
  5. pmc Huntingtin fragments and SOD1 mutants form soluble oligomers in the cell
    Yang Nim Park
    Laboratory of Cell Biology, National Heart Lung Blood Institute, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS ONE 7:e40329. 2012
  6. pmc B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy
    Isabella Palazzolo
    Neurogenetics Branch, NINDS, NIH, Bethesda, MD, USA
    J Neurosci Res 88:2207-16. 2010
  7. ncbi request reprint A screen for drugs that protect against the cytotoxicity of polyglutamine-expanded androgen receptor
    Federica Piccioni
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MS 20892 1250, USA
    Hum Mol Genet 13:437-46. 2004
  8. pmc Aberrant histone acetylation, altered transcription, and retinal degeneration in a Drosophila model of polyglutamine disease are rescued by CREB-binding protein
    J Paul Taylor
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 1250, USA
    Genes Dev 17:1463-8. 2003
  9. ncbi request reprint Hsp70 dynamics in vivo: effect of heat shock and protein aggregation
    Xian Chun Zeng
    Laboratory of Cell Biology, NHLBI, NIH, 50 South Drive MSC 8017, Bethesda, MD 20892 0301, USA
    J Cell Sci 117:4991-5000. 2004
  10. ncbi request reprint Valproic acid increases SMN levels in spinal muscular atrophy patient cells
    Charlotte J Sumner
    Neurogenetics Branch, National Institute of Neurologic Diseases and Stroke NIH, Building 10, Room 3B 14, MSC 1250, 10 Center Drive, Bethesda, MD 20892, USA
    Ann Neurol 54:647-54. 2003

Detail Information

Publications21

  1. ncbi request reprint 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...
  2. ncbi request reprint Altered acetylation in polyglutamine disease: an opportunity for therapeutic intervention?
    J Paul Taylor
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, Building 10, Room 3B 14, Bethesda, MD 20892 1250, USA
    Trends Mol Med 8:195-7. 2002
    ..These encouraging findings suggest that a novel strategy--pharmacological restoration of histone acetylation-- could prove effective in treating this group of devastating illnesses...
  3. ncbi request reprint Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein
    J Paul Taylor
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, Building 10, Room 3B 14, Bethesda, MD 20892 1250, USA
    Hum Mol Genet 12:749-57. 2003
    ..Together, these findings suggest that aggresomes are cytoprotective, serving as cytoplasmic recruitment centers to facilitate degradation of toxic proteins...
  4. pmc Overexpression of IGF-1 in muscle attenuates disease in a mouse model of spinal and bulbar muscular atrophy
    Isabella Palazzolo
    Neurogenetics Branch, NINDS, NIH, Bethesda, MD 20892, USA
    Neuron 63:316-28. 2009
    ..This study establishes IGF-1/Akt-mediated inactivation of mutant AR as a strategy to counteract disease in vivo and demonstrates that skeletal muscle is a viable target tissue for therapeutic intervention in SBMA...
  5. pmc Huntingtin fragments and SOD1 mutants form soluble oligomers in the cell
    Yang Nim Park
    Laboratory of Cell Biology, National Heart Lung Blood Institute, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS ONE 7:e40329. 2012
    ..Therefore, while soluble oligomers always seem to be present under conditions where cell pathology occurs, the presence of the oligomers, in itself, does not determine the extent of neuropathology...
  6. pmc B2 attenuates polyglutamine-expanded androgen receptor toxicity in cell and fly models of spinal and bulbar muscular atrophy
    Isabella Palazzolo
    Neurogenetics Branch, NINDS, NIH, Bethesda, MD, USA
    J Neurosci Res 88:2207-16. 2010
    ..Our findings suggest B2 as a novel approach to therapy for SBMA...
  7. ncbi request reprint A screen for drugs that protect against the cytotoxicity of polyglutamine-expanded androgen receptor
    Federica Piccioni
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MS 20892 1250, USA
    Hum Mol Genet 13:437-46. 2004
    ..The fourth compound, suloctidil, is a calcium channel blocker...
  8. pmc Aberrant histone acetylation, altered transcription, and retinal degeneration in a Drosophila model of polyglutamine disease are rescued by CREB-binding protein
    J Paul Taylor
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 1250, USA
    Genes Dev 17:1463-8. 2003
    ..These findings suggest that histone acetylation is an early target of polyglutamine toxicity and indicate that transcriptional dysregulation is an important part of the pathogenesis of polyglutamine-induced neurodegeneration...
  9. ncbi request reprint Hsp70 dynamics in vivo: effect of heat shock and protein aggregation
    Xian Chun Zeng
    Laboratory of Cell Biology, NHLBI, NIH, 50 South Drive MSC 8017, Bethesda, MD 20892 0301, USA
    J Cell Sci 117:4991-5000. 2004
    ..This provides strong evidence that Hsp70 interacts not only with organelles but also with diffusive proteins in the nucleoplasm and cytoplasm during heat shock as well as with diffusive huntingtin fragments...
  10. ncbi request reprint Valproic acid increases SMN levels in spinal muscular atrophy patient cells
    Charlotte J Sumner
    Neurogenetics Branch, National Institute of Neurologic Diseases and Stroke NIH, Building 10, Room 3B 14, MSC 1250, 10 Center Drive, Bethesda, MD 20892, USA
    Ann Neurol 54:647-54. 2003
    ..Valproic acid may increase SMN levels both by activating the SMN promoter and by preventing exon 7 skipping in SMN transcripts. Valproic acid and related compounds warrant further investigation as potential treatment for SMA...
  11. ncbi request reprint Rescue of polyglutamine-mediated cytotoxicity by double-stranded RNA-mediated RNA interference
    Natasha J Caplen
    Medical Genetics Branch, National Human Genome Research Institute and Neurogenetics Branch, National Institute of Neurological Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    Hum Mol Genet 11:175-84. 2002
    ..This study demonstrates the feasibility of targeting a transcript associated with an important group of genetic diseases by RNAi...
  12. pmc Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis
    Janel O Johnson
    1 Neuromuscular Diseases Research Section, Laboratory of Neurogenetics, National Institute on Aging, National Institutes of Health, Bethesda, Maryland, USA 2
    Nat Neurosci 17:664-6. 2014
    ..We also observed MATR3 pathology in ALS-affected spinal cords with and without MATR3 mutations. Our data provide more evidence supporting the role of aberrant RNA processing in motor neuron degeneration. ..
  13. pmc Mutational analysis of the VCP gene in Parkinson's disease
    Elisa Majounie
    Laboratory of Neurogenetics, National Institute of Aging, National Institutes of Health, Bethesda, MD, USA
    Neurobiol Aging 33:209.e1-2. 2012
    ..We identified a number of rare single nucleotide changes, including a variant previously described to be pathogenic, but no clear disease-causing variants. We conclude that mutations in VCP are not a common cause for idiopathic PD...
  14. ncbi request reprint Safety, tolerability, and pharmacokinetics of high-dose idebenone in patients with Friedreich ataxia
    Nicholas A Di Prospero
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 3705, USA
    Arch Neurol 64:803-8. 2007
    ..Some studies suggest that higher doses of idebenone may be more effective, but pharmacology and toxicology at higher doses have not been investigated in human beings...
  15. ncbi request reprint The role of autophagy in age-related neurodegeneration
    Brett A McCray
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Neurosignals 16:75-84. 2008
    ....
  16. ncbi request reprint 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
    ..Morover, these findings suggest that it may be possible to intervene in neurodegeneration by augmenting HDAC6 to enhance autophagy...
  17. ncbi request reprint Polyglutamines placed into context
    Albert R La Spada
    Department of Laboratory Medicine, Division of Medical Genetics Medicine University of Washington Medical Center, Seattle, WA 98195, USA
    Neuron 38:681-4. 2003
    ..Indeed, an intimate and inextricable relationship may exist between polyglutamine neurotoxicity and the normal interactions, domains, modifications, and functions of the respective disease proteins...
  18. pmc Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes
    Daniel J Klionsky
    Life Sciences Institute, University of Michigan, Ann Arbor, Michigan 48109 2216, USA
    Autophagy 4:151-75. 2008
    ..In addition, we emphasize that no individual assay is guaranteed to be the most appropriate one in every situation, and we strongly recommend the use of multiple assays to verify an autophagic response...
  19. ncbi request reprint HDAC6 at the intersection of autophagy, the ubiquitin-proteasome system and neurodegeneration
    Udai Bhan Pandey
    Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Autophagy 3:643-5. 2007
    ..Our findings indicate that HDAC6 facilitates degradation of potentially noxious protein substrates, contributing vitally to the neuroprotective role of autophagy...
  20. ncbi request reprint TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations
    Manuela Neumann
    Center for Neuropathology and Prion Research, Ludwig Maximilians University, Munich, Germany
    J Neuropathol Exp Neurol 66:152-7. 2007
    ..TDP-43 is a common pathologic substrate linking a variety of distinct patterns of FTLD-U pathology caused by different genetic alterations...
  21. ncbi request reprint Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy
    Jake B Guinto
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 422 Curie Blvd, 605B Stellar Chance Building, Philadelphia, PA, 19104 6140, USA
    Acta Neuropathol 114:55-61. 2007
    ..In this report, we review current literature on IBMPFD, focusing on the pathology of the disease and the biology of VCP with respect to IBMPFD...