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@ARTICLE{Jeibmann:120468,
      author       = {A. Jeibmann and J. Schulz and K. Eikmeier and P. Johann$^*$
                      and K. Thiel and I. Tegeder and O. Ambrée and M. C.
                      Frühwald and S. Pfister$^*$ and M. Kool$^*$ and W. Paulus
                      and M. Hasselblatt},
      title        = {{SMAD} dependent signaling plays a detrimental role in a
                      fly model of {SMARCB}1-deficiency and the biology of
                      atypical teratoid/rhabdoid tumors.},
      journal      = {Journal of neuro-oncology},
      volume       = {131},
      number       = {3},
      issn         = {1573-7373},
      address      = {Dordrecht [u.a.]},
      publisher    = {Springer Science + Business Media B.V},
      reportid     = {DKFZ-2017-00897},
      pages        = {477 - 484},
      year         = {2017},
      abstract     = {Atypical teratoid/rhabdoid tumors (ATRT) are highly
                      malignant brain tumors arising in young children. The
                      majority of ATRT is characterized by inactivation of the
                      chromatin remodeling complex member SMARCB1 (INI1/hSNF5).
                      Little is known, however, on downstream pathways involved in
                      the detrimental effects of SMARCB1 deficiency which might
                      also represent targets for treatment. Using Drosophila
                      melanogaster and the Gal4-UAS system, modifier screens were
                      performed in order to identify the role of SMAD dependent
                      signaling in the lethal phenotype associated with knockdown
                      of snr1, the fly homolog of SMARCB1. Expression and
                      functional role of human homologs was next investigated in
                      ATRT tumor samples and SMARCB1-deficient rhabdoid tumor
                      cells. The lethal phenotype associated with snr1 knockdown
                      in Drosophila melanogaster could be shifted to later stages
                      of development upon additional knockdown of several
                      decapentaplegic pathway members including Smox, and Med.
                      Similarly, the transforming growth factor beta (TGFbeta)
                      receptor type I kinase inhibitor SB431542 ameliorated the
                      detrimental effect of snr1 knockdown in the fruit fly.
                      Examination of homologs of candidate decapentaplegic pathway
                      members in human SMARCB1-deficent ATRT samples revealed
                      SMAD3 and SMAD6 to be over-expressed. In SMARCB1-deficent
                      rhabdoid tumor cells, siRNA-mediated silencing of SMAD3 or
                      SMAD6 expression reduced TGFbeta signaling activity and
                      resulted in decreased proliferation. Similar results were
                      obtained upon pharmacological inhibition of TGFbeta
                      signaling using SB431542. Our data suggest that SMAD
                      dependent signaling is involved in the detrimental effects
                      of SMARCB1-deficiency and provide a rationale for the
                      investigation of TGFbeta targeted treatments in ATRT.},
      cin          = {B062 / L101},
      ddc          = {610},
      cid          = {I:(DE-He78)B062-20160331 / I:(DE-He78)L101-20160331},
      pnm          = {312 - Functional and structural genomics (POF3-312)},
      pid          = {G:(DE-HGF)POF3-312},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:28108836},
      doi          = {10.1007/s11060-016-2326-3},
      url          = {https://inrepo02.dkfz.de/record/120468},
}