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@ARTICLE{Mller:281737,
      author       = {Möller, Meret and Nieweler, Johanna A and Nikulin, Vadim V
                      and van Riesen, Christoph},
      title        = {{N}etwork mechanisms in rapid-onset dystonia-parkinsonism.},
      journal      = {Experimental neurology},
      volume       = {395},
      issn         = {0014-4886},
      address      = {Amsterdam [u.a.]},
      publisher    = {Elsevier},
      reportid     = {DZNE-2025-01170},
      pages        = {115491},
      year         = {2025},
      abstract     = {Rapid-onset dystonia-parkinsonism (RDP) is a rare
                      neurological disorder caused by mutations in the ATP1A3
                      gene. Symptoms are characterized by a dystonia-parkinsonism.
                      Recently, experimental studies have shown that the
                      pathophysiology of the disease is based on a combined
                      dysfunction of the cerebellum (CB) and basal ganglia (BG)
                      and that blocking their interaction can alleviate the
                      symptoms. The underlying network mechanisms have not been
                      studied so far. Our aim was to characterize neuronal network
                      activity in the BG and CB and motor cortex in the ouabain
                      model of RDP by site-specific infusion of ouabain. Rats were
                      chronically infused with ouabain either in the CB, striatum
                      (STR) or at both places simultaneously. Motor behavior was
                      scored using published rating systems. Parallel in vivo
                      recordings of local field potentials (LFP) from M1, deep
                      cerebellar nuclei (DCN) and substantia nigra reticulata
                      (SNr) were performed. Data were compared to untreated
                      controls. Ouabain infusion into the cerebellum produced
                      severe dystonia that was associated with increased
                      high-frequency gamma oscillations in the DCNs, which were
                      subsequently transmitted to the BG and M1. Striatal infusion
                      led to parkinsonism and elevated beta-oscillations in SNr
                      that were transmitted to the CB and M1. The simultaneous
                      application of STRs and CB with ouabain resulted in
                      dystonia-parkinsonism and increased beta oscillations in BG,
                      CB, and M1. We demonstrate that symptom-specific beta and
                      gamma oscillations can be transmitted between the BG and CB,
                      which is likely to be very important for the understanding
                      of disease mechanisms.},
      keywords     = {Beta oscillations (Other) / Gamma oscillations (Other) /
                      Rapid-onset dystonia parkinsonism (Other)},
      cin          = {AG Fischer},
      ddc          = {610},
      cid          = {I:(DE-2719)1410002},
      pnm          = {352 - Disease Mechanisms (POF4-352)},
      pid          = {G:(DE-HGF)POF4-352},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:41047113},
      doi          = {10.1016/j.expneurol.2025.115491},
      url          = {https://pub.dzne.de/record/281737},
}