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@ARTICLE{Szewczyk:280968,
      author       = {Szewczyk, Barbara and Zimyanin, Vitaly and Japtok, Julia
                      and Held, Aaron and Pal, Arun and Großmann, Dajana and
                      Glaß, Hannes and Jürs, Alexandra V and Dash, Banaja P and
                      Bak, Maciek and Naumann, Marcel and Hartmann, Christiane and
                      Kuksenko, Olena and Günther, René and Kao, Tzu-Ting and
                      Sameith, Katrin and Dahl, Andreas and Sterneckert, Jared and
                      Aronica, Eleonora and Shneider, Neil A and Büttner, Andreas
                      and Catanese, Alberto and Phatnani, Hemali and Kipp, Markus
                      and Wainger, Brian J and Goswami, Anand and Hermann,
                      Andreas},
      title        = {{A}ctivation of polo-like kinase 1 correlates with
                      selective motor neuron vulnerability in familial {ALS}.},
      journal      = {Cell reports},
      volume       = {44},
      number       = {9},
      issn         = {2211-1247},
      address      = {Maryland Heights, MO},
      publisher    = {Cell Press},
      reportid     = {DZNE-2025-01050},
      pages        = {116113},
      year         = {2025},
      abstract     = {Mutations in the Fused in Sarcoma (FUS) gene cause familial
                      amyotrophic lateral sclerosis (ALS), characterized by
                      selective degeneration of spinal motor neurons (sMNs) with
                      relative sparing of cortical neurons (CNs). The mechanisms
                      underlying this cell-type vulnerability remain unclear.
                      Here, we compare CNs and sMNs derived from FUS-ALS models to
                      assess differential responses to FUS mutations. We find that
                      CNs are less affected than sMNs in DNA damage repair, axonal
                      organelle trafficking, and stress granule dynamics. RNA
                      sequencing (RNA-seq) reveals distinct transcriptomic
                      signatures, with sMNs uniquely activating DNA damage
                      responses involving cell cycle regulators, particularly
                      polo-like kinase 1 (PLK1). PLK1 is highly expressed in sMNs
                      but not CNs, correlating with greater nuclear FUS loss and
                      splicing defects in sMNs. Cross-comparison with other
                      familial ALS RNA-seq datasets highlights PLK1 upregulation
                      as a shared molecular feature. These findings identify
                      intrinsic differences between CNs and sMNs in FUS-ALS and
                      suggest PLK1 as a potential driver of sMN vulnerability.},
      keywords     = {CP: Molecular biology (Other) / CP: Neuroscience (Other) /
                      DNA damage response (Other) / FUS loss of function (Other) /
                      FUS-ALS (Other) / PLK1 (Other) / neurodegeneration (Other) /
                      polo-like kinase 1 (Other) / selective vulnerability (Other)
                      / transcriptomics (Other)},
      cin          = {AG Hermann},
      ddc          = {610},
      cid          = {I:(DE-2719)1511100},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:40857153},
      doi          = {10.1016/j.celrep.2025.116113},
      url          = {https://pub.dzne.de/record/280968},
}