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@ARTICLE{Bauer:247529,
      author       = {Bauer, Susanne and Chen, Chwen-Yu and Jonson, Maria and
                      Kaczmarczyk, Lech and Magadi, Srivathsa Subramanya and
                      Jackson, Walker Scot},
      title        = {{C}erebellar granule neurons induce {C}yclin {D}1 before
                      the onset of motor symptoms in {H}untington's disease mice.},
      journal      = {Acta Neuropathologica Communications},
      volume       = {11},
      number       = {1},
      issn         = {2051-5960},
      address      = {London},
      publisher    = {Biomed Central},
      reportid     = {DZNE-2023-00206},
      pages        = {17},
      year         = {2023},
      note         = {LIC: CCBY4},
      abstract     = {Although Huntington's disease (HD) is classically defined
                      by the selective vulnerability of striatal projection
                      neurons, there is increasing evidence that cerebellar
                      degeneration modulates clinical symptoms. However, little is
                      known about cell type-specific responses of cerebellar
                      neurons in HD. To dissect early disease mechanisms in the
                      cerebellum and cerebrum, we analyzed translatomes of
                      neuronal cell types from both regions in a new HD mouse
                      model. For this, HdhQ200 knock-in mice were backcrossed with
                      the calm 129S4 strain, to constrain experimental noise
                      caused by variable hyperactivity of mice in a C57BL/6
                      background. Behavioral and neuropathological
                      characterization showed that these S4-HdhQ200 mice had very
                      mild behavioral abnormalities starting around 12 months of
                      age that remained mild up to 18 months. By 9 months, we
                      observed abundant Huntingtin-positive neuronal intranuclear
                      inclusions (NIIs) in the striatum and cerebellum. The
                      translatome analysis of GABAergic cells of the cerebrum
                      further confirmed changes typical of HD-induced striatal
                      pathology. Surprisingly, we observed the strongest response
                      with 626 differentially expressed genes in glutamatergic
                      neurons of the cerebellum, a population consisting primarily
                      of granule cells, commonly considered disease resistant. Our
                      findings suggest vesicular fusion and exocytosis, as well as
                      differentiation-related pathways are affected in these
                      neurons. Furthermore, increased expression of cyclin D1
                      (Ccnd1) in the granular layer and upregulated expression of
                      polycomb group complex protein genes and cell cycle
                      regulators Cbx2, Cbx4 and Cbx8 point to a putative role of
                      aberrant cell cycle regulation in cerebellar granule cells
                      in early disease.},
      keywords     = {Mice / Animals / Huntington Disease: metabolism / Cyclin
                      D1: metabolism / Mice, Inbred C57BL / Interneurons:
                      pathology / Neurons: metabolism / Corpus Striatum / Disease
                      Models, Animal / Mice, Transgenic / Huntingtin Protein:
                      genetics / Huntingtin Protein: metabolism / Cyclin D1 (NLM
                      Chemicals) / Huntingtin Protein (NLM Chemicals)},
      cin          = {AG Jackson},
      ddc          = {610},
      cid          = {I:(DE-2719)1013019},
      pnm          = {352 - Disease Mechanisms (POF4-352)},
      pid          = {G:(DE-HGF)POF4-352},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:36670467},
      pmc          = {pmc:PMC9854201},
      doi          = {10.1186/s40478-022-01500-x},
      url          = {https://pub.dzne.de/record/247529},
}