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@ARTICLE{Kwon:282755,
      author       = {Y. Kwon and J. Kim and S.-Y. Cho and Y. J. Kang and J. Lee
                      and J. Kwon and H. Rhee and S. Bauer$^*$ and H.-S. Kim and
                      E. Lee and H. S. Kim and J. H. Jung and H. Kim and W. K.
                      Kim},
      title        = {{I}dentification of novel pathogenic roles of
                      {BLZF}1/{ATF}6 in tumorigenesis of gastrointestinal stromal
                      tumor showing {G}olgi-localized mutant {KIT}.},
      journal      = {Cell death and differentiation},
      volume       = {30},
      number       = {10},
      issn         = {1350-9047},
      address      = {London},
      publisher    = {Macmillan},
      reportid     = {DKFZ-2023-01874},
      pages        = {2309-2321},
      year         = {2023},
      note         = {2023 Oct;30(10):2309-2321},
      abstract     = {Gastrointestinal stromal tumors (GISTs) frequently show KIT
                      mutations, accompanied by overexpression and aberrant
                      localization of mutant KIT (MT-KIT). As previously
                      established by multiple studies, including ours, we
                      confirmed that MT-KIT initiates downstream signaling in the
                      Golgi complex. Basic leucine zipper nuclear factor 1 (BLZF1)
                      was identified as a novel MT-KIT-binding partner that
                      tethers MT-KIT to the Golgi complex. Sustained activation of
                      activated transcription factor 6 (ATF6), which belongs to
                      the unfolded protein response (UPR) family, alleviates
                      endoplasmic reticulum (ER) stress by upregulating chaperone
                      expression, including heat shock protein 90 (HSP90), which
                      assists in MT-KIT folding. BLZF1 knockdown and ATF6
                      inhibition suppressed both imatinib-sensitive and -resistant
                      GIST in vitro. ATF6 inhibitors further showed potent
                      antitumor effects in GIST xenografts, and the effect was
                      enhanced with ER stress-inducing drugs. ATF6 activation was
                      frequently observed in $67\%$ of patients with GIST (n =
                      42), and was significantly associated with poorer
                      relapse-free survival (P = 0.033). Overall, GIST bypasses ER
                      quality control (QC) and ER stress-mediated cell death via
                      UPR activation and uses the QC-free Golgi to initiate
                      signaling.},
      cin          = {ED01},
      ddc          = {610},
      cid          = {I:(DE-He78)ED01-20160331},
      pnm          = {899 - ohne Topic (POF4-899)},
      pid          = {G:(DE-HGF)POF4-899},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:37704840},
      doi          = {10.1038/s41418-023-01220-2},
      url          = {https://inrepo02.dkfz.de/record/282755},
}