Nicholas A DiProspero

Summary

Affiliation: National Institutes of Health
Country: USA

Publications

  1. ncbi request reprint Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial
    Nicholas A Di Prospero
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 3705, USA
    Lancet Neurol 6:878-86. 2007
  2. ncbi request reprint Early changes in Huntington's disease patient brains involve alterations in cytoskeletal and synaptic elements
    Nicholas A DiProspero
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, NIH, 9000 Rockville Pike, Bethesda, MD 20892, USA
    J Neurocytol 33:517-33. 2004
  3. ncbi request reprint Therapeutics development for triplet repeat expansion diseases
    Nicholas A Di Prospero
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892 3705, USA
    Nat Rev Genet 6:756-65. 2005
  4. 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
  5. ncbi request reprint PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains
    Jan Modregger
    Institute for Biochemistry II, University of Cologne, D 50931 Cologne, Germany
    Hum Mol Genet 11:2547-58. 2002
  6. 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
  7. pmc Phosphorylation of profilin by ROCK1 regulates polyglutamine aggregation
    Jieya Shao
    Departments of Neurology and Cellular and Molecular Pharmacology, UCSF, San Francisco, California 94143, USA
    Mol Cell Biol 28:5196-208. 2008

Collaborators

  • Kenneth H Fischbeck
  • J Paul Taylor
  • Scott R Penzak
  • Bernard Ravina
  • C J Sumner
  • E H Baehrecke
  • Nicholas A Di Prospero
  • Jieya Shao
  • Udai Bhan Pandey
  • Jan Modregger
  • Marc I Diamond
  • William J Welch
  • Marc Hild
  • Zhiping Nie
  • Melanie A Knight
  • Angela Baker
  • Natalia B Nedelsky
  • Brett A McCray
  • Ranjani Padmanabhan
  • Stephanie L Schwartz
  • Deborah L Berry
  • Gillian P Ritson
  • Neal Jeffries
  • Dan Garza
  • Charlotte C Hubbert
  • Tso Pang Yao
  • Yakup Batlevi
  • Oren Schuldiner
  • Danilo A Tagle
  • Markus Plomann
  • Vinod Charles

Detail Information

Publications7

  1. ncbi request reprint Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial
    Nicholas A Di Prospero
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 3705, USA
    Lancet Neurol 6:878-86. 2007
    ..Previous studies have indicated that the antioxidant idebenone (5 mg/kg daily) reduces cardiac hypertrophy, but definite improvement in neurological function has not been shown...
  2. ncbi request reprint Early changes in Huntington's disease patient brains involve alterations in cytoskeletal and synaptic elements
    Nicholas A DiProspero
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, NIH, 9000 Rockville Pike, Bethesda, MD 20892, USA
    J Neurocytol 33:517-33. 2004
    ..We propose that mutant huntingtin affects proteins involved in synaptic function and cytoskeletal integrity before symptoms develop which may influence early disease onset and/or progression...
  3. ncbi request reprint Therapeutics development for triplet repeat expansion diseases
    Nicholas A Di Prospero
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892 3705, USA
    Nat Rev Genet 6:756-65. 2005
    ..Examination of experimental findings reveals similarities in disease mechanisms as well as possibilities for treatment...
  4. 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...
  5. ncbi request reprint PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains
    Jan Modregger
    Institute for Biochemistry II, University of Cologne, D 50931 Cologne, Germany
    Hum Mol Genet 11:2547-58. 2002
    ....
  6. 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...
  7. pmc Phosphorylation of profilin by ROCK1 regulates polyglutamine aggregation
    Jieya Shao
    Departments of Neurology and Cellular and Molecular Pharmacology, UCSF, San Francisco, California 94143, USA
    Mol Cell Biol 28:5196-208. 2008
    ..A signaling pathway from ROCK1 to profilin thus controls polyglutamine protein aggregation and is targeted by a promising therapeutic lead for HD...