Barrington G Burnett

Summary

Affiliation: National Institutes of Health
Country: USA

Publications

  1. ncbi Regulation of SMN protein stability
    Barrington G Burnett
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Mol Cell Biol 29:1107-15. 2009
  2. ncbi Expression of expanded polyglutamine targets profilin for degradation and alters actin dynamics
    Barrington G Burnett
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, Maryland, USA
    Neurobiol Dis 30:365-74. 2008
  3. ncbi Sustained improvement of spinal muscular atrophy mice treated with trichostatin A plus nutrition
    Heather L Narver
    Animal Care Division, National Institute of Neurological Disorders and Stroke, National Institute of Health, Johns Hopkins University, Baltimore, MD 21287, USA
    Ann Neurol 64:465-70. 2008
  4. ncbi Mitochondrial abnormalities in spinal and bulbar muscular atrophy
    Srikanth Ranganathan
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, Bethesda, MD, USA
    Hum Mol Genet 18:27-42. 2009
  5. ncbi Histone deacetylase inhibition suppresses myogenin-dependent atrogene activation in spinal muscular atrophy mice
    Katherine V Bricceno
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA
    Hum Mol Genet 21:4448-59. 2012
  6. ncbi Increasing expression and decreasing degradation of SMN ameliorate the spinal muscular atrophy phenotype in mice
    Deborah Y Kwon
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD, USA
    Hum Mol Genet 20:3667-77. 2011
  7. ncbi Cowchock syndrome is associated with a mutation in apoptosis-inducing factor
    Carlo Rinaldi
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 3705, USA
    Am J Hum Genet 91:1095-102. 2012
  8. ncbi Neurogenic and myogenic contributions to hereditary motor neuron disease
    Katherine V Bricceno
    National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA
    Neurodegener Dis 9:199-209. 2012
  9. ncbi Akt blocks ligand binding and protects against expanded polyglutamine androgen receptor toxicity
    Isabella Palazzolo
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 35 Convent Drive, Bethesda, MD 20892, USA
    Hum Mol Genet 16:1593-603. 2007
  10. ncbi Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy
    Amy M Avila
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke NINDS, NIH, Bethesda, MA 20892, USA
    J Clin Invest 117:659-71. 2007

Collaborators

Detail Information

Publications13

  1. ncbi Regulation of SMN protein stability
    Barrington G Burnett
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892, USA
    Mol Cell Biol 29:1107-15. 2009
    ..Together, our data indicate that SMN protein stability is modulated by complex formation. Promotion of the SMN complex formation may be an important novel therapeutic strategy for SMA...
  2. ncbi Expression of expanded polyglutamine targets profilin for degradation and alters actin dynamics
    Barrington G Burnett
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, Maryland, USA
    Neurobiol Dis 30:365-74. 2008
    ..These results indicate a link between huntingtin and profilin and implicate profilin in Huntington's disease pathogenesis...
  3. ncbi Sustained improvement of spinal muscular atrophy mice treated with trichostatin A plus nutrition
    Heather L Narver
    Animal Care Division, National Institute of Neurological Disorders and Stroke, National Institute of Health, Johns Hopkins University, Baltimore, MD 21287, USA
    Ann Neurol 64:465-70. 2008
    ..Early spinal muscular atrophy disease detection and treatment initiation combined with aggressive ancillary care may be integral to the optimization of histone deacetylase inhibitor treatment in human patients...
  4. ncbi Mitochondrial abnormalities in spinal and bulbar muscular atrophy
    Srikanth Ranganathan
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, Bethesda, MD, USA
    Hum Mol Genet 18:27-42. 2009
    ..These findings indicate possible benefit from mitochondrial therapy for SBMA...
  5. ncbi Histone deacetylase inhibition suppresses myogenin-dependent atrogene activation in spinal muscular atrophy mice
    Katherine V Bricceno
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA
    Hum Mol Genet 21:4448-59. 2012
    ....
  6. ncbi Increasing expression and decreasing degradation of SMN ameliorate the spinal muscular atrophy phenotype in mice
    Deborah Y Kwon
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD, USA
    Hum Mol Genet 20:3667-77. 2011
    ..Moreover, this study indicates that while increasing SMN levels in the central nervous system may help extend survival, peripheral tissues can also be targeted to improve the SMA disease phenotype...
  7. ncbi Cowchock syndrome is associated with a mutation in apoptosis-inducing factor
    Carlo Rinaldi
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892 3705, USA
    Am J Hum Genet 91:1095-102. 2012
    ..Our findings expand the spectrum of AIF-related disease and provide insight into the effects of AIFM1 mutations...
  8. ncbi Neurogenic and myogenic contributions to hereditary motor neuron disease
    Katherine V Bricceno
    National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA
    Neurodegener Dis 9:199-209. 2012
    ..Characterizing these contributions helps us to better understand the disease mechanisms and to better target therapeutic intervention...
  9. ncbi Akt blocks ligand binding and protects against expanded polyglutamine androgen receptor toxicity
    Isabella Palazzolo
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 35 Convent Drive, Bethesda, MD 20892, USA
    Hum Mol Genet 16:1593-603. 2007
    ..IGF-1 rescue of AR toxicity is diminished by alanine substitutions at the Akt consensus sites. These results highlight potential targets for therapeutic intervention in SBMA...
  10. ncbi Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy
    Amy M Avila
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke NINDS, NIH, Bethesda, MA 20892, USA
    J Clin Invest 117:659-71. 2007
    ..These results indicate that the hydroxamic acid class of HDAC inhibitors activates SMN2 gene expression in vivo and has an ameliorating effect on the SMA disease phenotype when administered after disease onset...
  11. ncbi A candidate gene for autoimmune myasthenia gravis
    Guida Landoure
    Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA
    Neurology 79:342-7. 2012
    ..We sought to identify a causative mutation in a previously reported kindred with parental consanguinity and 5 of 10 siblings with adult-onset autoimmune myasthenia gravis...
  12. ncbi Targeting splicing in spinal muscular atrophy
    Barrington G Burnett
    Ann Neurol 63:3-6. 2008
  13. 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
    ..These findings indicate that miRNA pathways dramatically modulate polyQ- and tau-induced neurodegeneration, providing the foundation for new insight into therapeutics...