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@ARTICLE{Kutscher:157608,
      author       = {L. Kutscher$^*$ and K. Okonechnikov$^*$ and N. Batora and
                      J. Clark$^*$ and P. B. G. Silva$^*$ and M. Vouri$^*$ and S.
                      van Rijn$^*$ and L. Sieber$^*$ and B. Statz$^*$ and M. D.
                      Gearhart and R. Shiraishi and N. Mack$^*$ and B. A. Orr and
                      A. Korshunov$^*$ and B. L. Gudenas and K. S. Smith and A. L.
                      Mercier and O. Ayrault and M. Hoshino and M. Kool$^*$ and K.
                      von Hoff and N. Graf and G. Fleishhack and V. J.
                      Bardwell$^*$ and S. M. Pfister$^*$ and P. A. Northcott and
                      D. Kawauchi$^*$},
      title        = {{F}unctional loss of a noncanonical {BCOR}-{PRC}1.1 complex
                      accelerates {SHH}-driven medulloblastoma formation.},
      journal      = {Genes $\&$ development},
      volume       = {34},
      number       = {17-18},
      issn         = {1549-5477},
      address      = {Cold Spring Harbor, NY},
      publisher    = {Laboratory Press},
      reportid     = {DKFZ-2020-01705},
      pages        = {1161-1176},
      year         = {2020},
      note         = {2020 Sep 1;34(17-18):1161-1176#EA:B062#LA:B062#},
      abstract     = {Medulloblastoma is a malignant childhood brain tumor
                      arising from the developing cerebellum. In Sonic Hedgehog
                      (SHH) subgroup medulloblastoma, aberrant activation of SHH
                      signaling causes increased proliferation of granule neuron
                      progenitors (GNPs), and predisposes these cells to
                      tumorigenesis. A second, cooperating genetic hit is often
                      required to push these hyperplastic cells to malignancy and
                      confer mutation-specific characteristics associated with
                      oncogenic signaling. Somatic loss-of-function mutations of
                      the transcriptional corepressor BCOR are recurrent and
                      enriched in SHH medulloblastoma. To investigate BCOR as a
                      putative tumor suppressor, we used a genetically engineered
                      mouse model to delete exons 9/10 of Bcor (BcorΔE9-10 ) in
                      GNPs during development. This mutation leads to reduced
                      expression of C-terminally truncated BCOR (BCORΔE9-10).
                      While BcorΔE9-10 alone did not promote tumorigenesis or
                      affect GNP differentiation, BcorΔE9-10 combined with loss
                      of the SHH receptor gene Ptch1 resulted in fully penetrant
                      medulloblastomas. In Ptch1+/-;BcorΔE9-10 tumors, the growth
                      factor gene Igf2 was aberrantly up-regulated, and ectopic
                      Igf2 overexpression was sufficient to drive tumorigenesis in
                      Ptch1+/- GNPs. BCOR directly regulates Igf2, likely through
                      the PRC1.1 complex; the repressive histone mark H2AK119Ub is
                      decreased at the Igf2 promoter in Ptch1+/-;BcorΔE9-10
                      tumors. Overall, our data suggests that BCOR-PRC1.1
                      disruption leads to Igf2 overexpression, which transforms
                      preneoplastic cells to malignant tumors.},
      cin          = {B062 / HD01 / B300},
      ddc          = {570},
      cid          = {I:(DE-He78)B062-20160331 / I:(DE-He78)HD01-20160331 /
                      I:(DE-He78)B300-20160331},
      pnm          = {312 - Functional and structural genomics (POF3-312)},
      pid          = {G:(DE-HGF)POF3-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:32820036},
      doi          = {10.1101/gad.337584.120},
      url          = {https://inrepo02.dkfz.de/record/157608},
}