Vanessa Wheeler

Summary

Affiliation: Harvard University
Country: USA

Publications

  1. pmc A novel approach to investigate tissue-specific trinucleotide repeat instability
    Jong Min Lee
    Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA, USA
    BMC Syst Biol 4:29. 2010
  2. pmc Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study
    Jian Liang Li
    Department of Neurology, Boston University School of Medicine, Boston, MA, USA
    BMC Med Genet 7:71. 2006
  3. pmc Factors associated with HD CAG repeat instability in Huntington disease
    V C Wheeler
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    J Med Genet 44:695-701. 2007
  4. ncbi request reprint Mismatch repair gene Msh2 modifies the timing of early disease in Hdh(Q111) striatum
    Vanessa C Wheeler
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown, MA 02129, USA
    Hum Mol Genet 12:273-81. 2003
  5. pmc Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes
    Ella Dragileva
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Boston MA 02114, USA
    Neurobiol Dis 33:37-47. 2009
  6. ncbi request reprint Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice
    Vanessa C Wheeler
    Molecular Neurogenetics Unit and Molecular Neuropathology, Massachusetts General Hospital, Charlestown, MA 02129, USA
    Hum Mol Genet 11:633-40. 2002
  7. ncbi request reprint Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice
    Alejandro Lloret
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    Hum Mol Genet 15:2015-24. 2006
  8. ncbi request reprint The HD mutation does not alter neuronal death in the striatum of Hdh(Q92) knock-in mice after mild focal ischemia
    Shobu Namura
    Stroke and Neurovascular Regulation, Massachusetts General Hospital, Charlestown, 02129, USA
    Neurobiol Dis 11:147-54. 2002
  9. pmc Huntingtin facilitates polycomb repressive complex 2
    Ihn Sik Seong
    Center for Human Genetic Research, Massachusetts General Hospital, 185 Cambridge Street, Boston, MA 02114, USA
    Hum Mol Genet 19:573-83. 2010
  10. ncbi request reprint Huntington's disease
    Marcy E MacDonald
    Molecular Neurogenetics Unit, Center for Human Genetics Research, Massachusetts General Hospital, Charlestown, MA 02129, USA
    Neuromolecular Med 4:7-20. 2003

Collaborators

Detail Information

Publications16

  1. pmc A novel approach to investigate tissue-specific trinucleotide repeat instability
    Jong Min Lee
    Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA, USA
    BMC Syst Biol 4:29. 2010
    ..However progress in this area has been hampered by the lack of sensitive high-throughput instability quantification methods and global approaches to identify the underlying factors...
  2. pmc Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study
    Jian Liang Li
    Department of Neurology, Boston University School of Medicine, Boston, MA, USA
    BMC Med Genet 7:71. 2006
    ....
  3. pmc Factors associated with HD CAG repeat instability in Huntington disease
    V C Wheeler
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    J Med Genet 44:695-701. 2007
    ..The instability of the HD disease allele in male intergenerational transmissions is reflected in the variability of the CAG repeat in DNA from the sperm of male carriers of the HD gene...
  4. ncbi request reprint Mismatch repair gene Msh2 modifies the timing of early disease in Hdh(Q111) striatum
    Vanessa C Wheeler
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown, MA 02129, USA
    Hum Mol Genet 12:273-81. 2003
    ..Therefore Msh2, as a striking modifier of early disease onset in a precise genetic HD mouse model, provides a novel target for the development of pharmacological agents that aim to slow pathogenesis in man...
  5. pmc Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes
    Ella Dragileva
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Boston MA 02114, USA
    Neurobiol Dis 33:37-47. 2009
    ....
  6. ncbi request reprint Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice
    Vanessa C Wheeler
    Molecular Neurogenetics Unit and Molecular Neuropathology, Massachusetts General Hospital, Charlestown, MA 02129, USA
    Hum Mol Genet 11:633-40. 2002
    ..Thus, potential HD therapies in man might be directed at different levels: preventing the disease-initiating mechanism or slowing the subsequent progression of pathogenesis...
  7. ncbi request reprint Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice
    Alejandro Lloret
    Molecular Neurogenetics Unit, Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    Hum Mol Genet 15:2015-24. 2006
    ..Our findings set the stage for defining disease-related genetic pathways that will ultimately provide insight into disease mechanism...
  8. ncbi request reprint The HD mutation does not alter neuronal death in the striatum of Hdh(Q92) knock-in mice after mild focal ischemia
    Shobu Namura
    Stroke and Neurovascular Regulation, Massachusetts General Hospital, Charlestown, 02129, USA
    Neurobiol Dis 11:147-54. 2002
    ..However, they suggest that calpain activation and huntingtin regulation merit investigation as modifiers of disease progression in neurons injured by the harmful consequences of full-length mutant huntingtin...
  9. pmc Huntingtin facilitates polycomb repressive complex 2
    Ihn Sik Seong
    Center for Human Genetic Research, Massachusetts General Hospital, 185 Cambridge Street, Boston, MA 02114, USA
    Hum Mol Genet 19:573-83. 2010
    ....
  10. ncbi request reprint Huntington's disease
    Marcy E MacDonald
    Molecular Neurogenetics Unit, Center for Human Genetics Research, Massachusetts General Hospital, Charlestown, MA 02129, USA
    Neuromolecular Med 4:7-20. 2003
    ....
  11. ncbi request reprint Identification of a presymptomatic molecular phenotype in Hdh CAG knock-in mice
    Elisa Fossale
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Building 149, 13th Street, Charlestown, MA 02129, USA
    Hum Mol Genet 11:2233-41. 2002
    ..These findings, therefore, support a chronic impact of mutant huntingtin on an essential ribosomal regulatory gene to be investigated for its role very early in HD pathogenesis...
  12. pmc Estimating the probability of de novo HD cases from transmissions of expanded penetrant CAG alleles in the Huntington disease gene from male carriers of high normal alleles (27-35 CAG)
    Audrey E Hendricks
    Department of Biostatistics, Boston University School of Public Health, Boston, Massachusetts 02118, USA
    Am J Med Genet A 149:1375-81. 2009
    ..These estimates may be useful in genetic counseling for male high normal allele carriers...
  13. ncbi request reprint Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice
    Silvia Gines
    Molecular Neurogenetics Unit, Massachusetts General Hospital, Building 149, 13th St, Charlestown, MA 02129, USA
    Hum Mol Genet 12:497-508. 2003
    ....
  14. pmc A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study
    Jian Liang Li
    Department of Neurology, Boston University School of Medicine, and Bioinformatics Program, School of Public Health, Boston University, Boston, MA, USA
    Am J Hum Genet 73:682-7. 2003
    ..Suggestive evidence for linkage was found at 4p16 (LOD=1.93), 6p21-23 (LOD=2.29), and 6q24-26 (LOD=2.28), which may be useful for investigation of genes that modify age at onset of HD...
  15. pmc Somatic expansion of the Huntington's disease CAG repeat in the brain is associated with an earlier age of disease onset
    Meera Swami
    Center for Human Genetic Research, Massachusetts General Hospital, Boston, MA 02114, USA
    Hum Mol Genet 18:3039-47. 2009
    ....
  16. ncbi request reprint Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice
    Paolo Guidetti
    Maryland Psychiatric Research Center, University of Maryland School of Medicine, Baltimore, MD 21228, USA
    Neurobiol Dis 23:190-7. 2006
    ....