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@ARTICLE{Siebzehnrbl:852456,
      author       = {Siebzehnrübl, Florian A. and Raber, Kerstin A. and Urbach,
                      Yvonne K. and Schulze-Krebs, Anja and Canneva, Fabio and
                      Moceri, Sandra and Habermeyer, Johanna and Achoui, Dalila
                      and Gupta, Bhavana and Steindler, Dennis A. and Stephan,
                      Michael and Nguyen, Huu Phuc and Bonin, Michael and Riess,
                      Olaf and Bauer, Andreas and Aigner, Ludwig and
                      Couillard-Despres, Sebastien and Paucar, Martin Arce and
                      Svenningsson, Per and Osmand, Alexander and Andreew,
                      Alexander and Zabel, Claus and Weiss, Andreas and Kuhn,
                      Rainer and Moussaoui, Saliha and Blockx, Ines and Van der
                      Linden, Annemie and Cheong, Rachel Y. and Roybon, Laurent
                      and Petersén, Åsa and von Hörsten, Stephan},
      title        = {{E}arly postnatal behavioral, cellular, and molecular
                      changes in models of {H}untington disease are reversible by
                      {HDAC} inhibition},
      journal      = {Proceedings of the National Academy of Sciences of the
                      United States of America},
      volume       = {115},
      number       = {37},
      issn         = {1091-6490},
      address      = {Washington, DC},
      publisher    = {National Acad. of Sciences},
      reportid     = {FZJ-2018-05402},
      pages        = {E8765 - E8774},
      year         = {2018},
      abstract     = {Huntington disease (HD) is an autosomal dominant
                      neurodegenerative disorder caused by expanded CAG repeats in
                      the huntingtin gene (HTT). Although mutant HTT is expressed
                      during embryonic development and throughout life, clinical
                      HD usually manifests later in adulthood. A number of studies
                      document neurodevelopmental changes associated with mutant
                      HTT, but whether these are reversible under therapy remains
                      unclear. Here, we identify very early behavioral, molecular,
                      and cellular changes in preweaning transgenic HD rats and
                      mice. Reduced ultrasonic vocalization, loss of prepulse
                      inhibition, and increased risk taking are accompanied by
                      disturbances of dopaminergic regulation in vivo, reduced
                      neuronal differentiation capacity in subventricular zone
                      stem/progenitor cells, and impaired neuronal and
                      oligodendrocyte differentiation of mouse embryo-derived
                      neural stem cells in vitro. Interventional treatment of this
                      early phenotype with the histone deacetylase inhibitor
                      (HDACi) LBH589 led to significant improvement in behavioral
                      changes and markers of dopaminergic neurotransmission and
                      complete reversal of aberrant neuronal differentiation in
                      vitro and in vivo. Our data support the notion that
                      neurodevelopmental changes contribute to the prodromal phase
                      of HD and that early, presymptomatic intervention using
                      HDACi may represent a promising novel treatment approach for
                      HD.},
      cin          = {INM-2},
      ddc          = {000},
      cid          = {I:(DE-Juel1)INM-2-20090406},
      pnm          = {573 - Neuroimaging (POF3-573)},
      pid          = {G:(DE-HGF)POF3-573},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:30150378},
      UT           = {WOS:000444257200022},
      doi          = {10.1073/pnas.1807962115},
      url          = {https://juser.fz-juelich.de/record/852456},
}