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@ARTICLE{Pschl:120154,
      author       = {J. Pöschl and S. Stark$^*$ and P. Neumann and S.
                      Gröbner$^*$ and D. Kawauchi$^*$ and D. Jones$^*$ and P. A.
                      Northcott$^*$ and P. Lichter$^*$ and S. Pfister$^*$ and M.
                      Kool$^*$ and U. Schüller},
      title        = {{G}enomic and transcriptomic analyses match medulloblastoma
                      mouse models to their human counterparts.},
      journal      = {Acta neuropathologica},
      volume       = {128},
      number       = {1},
      issn         = {1432-0533},
      address      = {Berlin},
      publisher    = {Springer},
      reportid     = {DKFZ-2017-00737},
      pages        = {123 - 136},
      year         = {2014},
      abstract     = {Medulloblastoma is a malignant embryonal brain tumor with
                      highly variable outcome. In order to study the biology of
                      this tumor and to perform preclinical treatment studies, a
                      lot of effort has been put into the generation of
                      appropriate mouse models. The usage of these models,
                      however, has become debatable with the advances in human
                      medulloblastoma subgrouping. This study brings together
                      multiple relevant mouse models and matches genetic
                      alterations and gene expression data of 140 murine tumors
                      with 423 human medulloblastomas in a global way. Using AGDEX
                      analysis and k-means clustering, we show that the
                      Blbp-cre::Ctnnb1(ex3)(Fl/+)Trp53 (Fl/Fl) mouse model fits
                      well to human WNT medulloblastoma, and that, among various
                      Myc- or Mycn-based mouse medulloblastomas, tumors in
                      Glt1-tTA::TRE-MYCN/Luc mice proved to be most specific for
                      human group 3 medulloblastoma. None of the analyzed models
                      displayed a significant match to group 4 tumors.
                      Intriguingly, mice with Ptch1 or Smo mutations selectively
                      modeled SHH medulloblastomas of adulthood, although such
                      mutations occur in all human age groups. We therefore
                      suggest that the infantile or adult gene expression pattern
                      of SHH MBs are not solely determined by specific mutations.
                      This is supported by the observation that human
                      medulloblastomas with PTCH1 mutations displayed more
                      similarities to PTCH1 wild-type tumors of the same age group
                      than to PTCH1-mutated tumors of the other age group.
                      Together, we provide novel insights into previously
                      unrecognized specificity of distinct models and suggest
                      these findings as a solid basis to choose the appropriate
                      model for preclinical studies on medulloblastoma.},
      keywords     = {Hedgehog Proteins (NLM Chemicals) / PTCH protein, human
                      (NLM Chemicals) / Patched Receptors (NLM Chemicals) /
                      Patched-1 Receptor (NLM Chemicals) / Ptch1 protein, mouse
                      (NLM Chemicals) / Receptors, Cell Surface (NLM Chemicals) /
                      SHH protein, human (NLM Chemicals)},
      cin          = {B062 / B060},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331 / I:(DE-He78)B060-20160331},
      pnm          = {312 - Functional and structural genomics (POF3-312)},
      pid          = {G:(DE-HGF)POF3-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:24871706},
      doi          = {10.1007/s00401-014-1297-8},
      url          = {https://inrepo02.dkfz.de/record/120154},
}