Targeting H3K4 trimethylation in Huntington disease

  • Malini Vashishtha
    Department of Psychiatry and Human Behavior and UCI Institute of Memory Impairments and Neurological Disorders,
  • Christopher W. Ng
    Department of Biological Engineering and
  • Ferah Yildirim
    Department of Biological Engineering and
  • Theresa A. Gipson
    Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, MA 02139;
  • Ian H. Kratter
    Gladstone Institute of Neurological Disease Biomedical Sciences Graduate Program and Medical Scientist Training Program and
  • Laszlo Bodai
    Department of Developmental and Cell Biology and Developmental Biology Center, and
  • Wan Song
    Department of Developmental and Cell Biology and Developmental Biology Center, and
  • Alice Lau
    Department of Psychiatry and Human Behavior and UCI Institute of Memory Impairments and Neurological Disorders,
  • Adam Labadorf
    Department of Biological Engineering and
  • Annie Vogel-Ciernia
    Department of Neurobiology and Behavior, University of California, Irvine, CA 92697;
  • Juan Troncosco
    Departments of Psychiatry, Neurology, and Neuroscience and Neuropathology, Johns Hopkins University, Baltimore, MD 21287;
  • Christopher A. Ross
    Departments of Psychiatry, Neurology, and Neuroscience and Neuropathology, Johns Hopkins University, Baltimore, MD 21287;
  • Gillian P. Bates
    Department of Medical and Molecular Genetics, King's College, London SE1 9RT, United Kingdom; and
  • Dimitri Krainc
    Department of Neurology, Massachusetts General Hospital, Boston, MA 02114
  • Ghazaleh Sadri-Vakili
    Department of Neurology, Massachusetts General Hospital, Boston, MA 02114
  • Steven Finkbeiner
    Gladstone Institute of Neurological Disease Taube-Koret Center for Huntington’s Disease Research, Departments of Neurology and Physiology, University of California, San Francisco, CA 94158;
  • J. Lawrence Marsh
    Department of Developmental and Cell Biology and Developmental Biology Center, and
  • David E. Housman
    Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, MA 02139;
  • Ernest Fraenkel
    Department of Biological Engineering and
  • Leslie M. Thompson
    Department of Psychiatry and Human Behavior and UCI Institute of Memory Impairments and Neurological Disorders,

説明

<jats:title>Significance</jats:title> <jats:p> Transcriptional dysregulation is an early and reproducible feature of Huntington disease (HD); however, mechanisms underlying this dysregulation are unclear. This article describes a unique pattern of the chromatin mark H3K4me3 at transcriptionally repressed promoters in HD mouse and human brain identified by genome-wide analysis. Reducing the levels of the demethylase <jats:italic>SMCX/Jarid1c</jats:italic> in primary neurons reversed down-regulation of key neuronal genes caused by mutant Huntingtin expression and was neuroprotective in a <jats:italic>Drosophila</jats:italic> HD model. These results suggest that targeting epigenetic signatures may be an effective strategy to ameliorate the consequences of HD and other neurodegenerative diseases. </jats:p>

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