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@ARTICLE{Paassen:275262,
      author       = {I. Paassen and J. Williams and C. Ríos Arceo and F.
                      Ringnalda and K. S. Mercer and J. L. Buhl and N. Moreno and
                      A. Federico$^*$ and N. E. Franke and M. Kranendonk and S. A.
                      Upadhyaya and K. Kerl and M. van de Wetering and H. Clevers
                      and M. Kool$^*$ and E. W. Hoving and M. F. Roussel and J.
                      Drost},
      title        = {{A}typical teratoid/rhabdoid tumoroids reveal
                      subgroup-specific drug vulnerabilities.},
      journal      = {Oncogene},
      volume       = {42},
      number       = {20},
      issn         = {0950-9232},
      address      = {London},
      publisher    = {Springer Nature},
      reportid     = {DKFZ-2023-00709},
      pages        = {1661-1671},
      year         = {2023},
      note         = {2023 May;42(20):1661-1671},
      abstract     = {Atypical teratoid/rhabdoid tumors (ATRTs) represent a rare,
                      but aggressive pediatric brain tumor entity. They are
                      genetically defined by alterations in the SWI/SNF chromatin
                      remodeling complex members SMARCB1 or SMARCA4. ATRTs can be
                      further classified in different molecular subgroups based on
                      their epigenetic profiles. Although recent studies suggest
                      that the different subgroups have distinct clinical
                      features, subgroup-specific treatment regimens have not been
                      developed thus far. This is hampered by the lack of
                      pre-clinical in vitro models representative of the different
                      molecular subgroups. Here, we describe the establishment of
                      ATRT tumoroid models from the ATRT-MYC and ATRT-SHH
                      subgroups. We demonstrate that ATRT tumoroids retain
                      subgroup-specific epigenetic and gene expression profiles.
                      High throughput drug screens on our ATRT tumoroids revealed
                      distinct drug sensitivities between and within ATRT-MYC and
                      ATRT-SHH subgroups. Whereas ATRT-MYC universally displayed
                      high sensitivity to multi-targeted tyrosine kinase
                      inhibitors, ATRT-SHH showed a more heterogeneous response
                      with a subset showing high sensitivity to NOTCH inhibitors,
                      which corresponded to high expression of NOTCH receptors.
                      Our ATRT tumoroids represent the first pediatric brain tumor
                      organoid model, providing a representative pre-clinical
                      model which enables the development of subgroup-specific
                      therapies.},
      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:37020038},
      doi          = {10.1038/s41388-023-02681-y},
      url          = {https://inrepo02.dkfz.de/record/275262},
}