% IMPORTANT: The following is UTF-8 encoded.  This means that in the presence
% of non-ASCII characters, it will not work with BibTeX 0.99 or older.
% Instead, you should use an up-to-date BibTeX implementation like “bibtex8” or
% “biber”.

@ARTICLE{Metselaar:292537,
      author       = {D. S. Metselaar and M. H. Meel and J. R. Goulding and A. du
                      Chatinier and L. Rigamonti and P. Waranecki and N.
                      Geisemeyer$^*$ and M. C. de Gooijer and M. Breur and J.
                      Koster and S. E. M. Veldhuijzen van Zanten and M. Bugiani
                      and N. E. Franke and A. Reddy and P. Wesseling and G. J. L.
                      Kaspers and E. Hulleman},
      title        = {{G}emcitabine therapeutically disrupts essential
                      {SIRT}1-mediated p53 repression in atypical
                      teratoid/rhabdoid tumors.},
      journal      = {Cell reports / Medicine},
      volume       = {5},
      number       = {9},
      issn         = {2666-3791},
      address      = {Maryland Heights, MO},
      publisher    = {Elsevier},
      reportid     = {DKFZ-2024-01780},
      pages        = {101700},
      year         = {2024},
      note         = {2024 Sep 17;5(9):101700},
      abstract     = {Atypical teratoid/rhabdoid tumors (ATRTs) are highly
                      malignant embryonal tumors of the central nervous system
                      with a dismal prognosis. Using a newly developed and
                      validated patient-derived ATRT culture and xenograft model,
                      alongside a panel of primary ATRT models, we found that
                      ATRTs are selectively sensitive to the nucleoside analog
                      gemcitabine. Gene expression and protein analyses indicate
                      that gemcitabine treatment causes the degradation of sirtuin
                      1 (SIRT1), resulting in cell death through activation of
                      nuclear factor κB (NF-κB) and p53. Furthermore, we
                      discovered that gemcitabine-induced loss of SIRT1 results in
                      a nucleus-to-cytoplasm translocation of the sonic hedgehog
                      (SHH) signaling activator GLI2, explaining the observed
                      additional gemcitabine sensitivity in SHH-subtype ATRT.
                      Treatment of ATRT xenograft-bearing mice with gemcitabine
                      resulted in a $>30\%$ increase in median survival and
                      yielded long-term survivors in two independent
                      patient-derived xenograft models. These findings demonstrate
                      that ATRTs are highly sensitive to gemcitabine treatment and
                      may form part of a future multimodal treatment strategy for
                      ATRTs.},
      keywords     = {ATRT (Other) / atypical teratoid/rhabdoid tumor (Other) /
                      gemcitabine (Other) / neuro oncology (Other) / p53 (Other) /
                      patient-derived models (Other) / pediatric oncology (Other)
                      / sirtuin 1 (Other) / therapy development (Other)},
      cin          = {B062 / HD01},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331 / I:(DE-He78)HD01-20160331},
      pnm          = {312 - Funktionelle und strukturelle Genomforschung
                      (POF4-312)},
      pid          = {G:(DE-HGF)POF4-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:39208799},
      doi          = {10.1016/j.xcrm.2024.101700},
      url          = {https://inrepo02.dkfz.de/record/292537},
}