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@ARTICLE{Duerig:278369,
      author       = {I. Duerig and E. Pylaeva and I. Ozel and S. Weinwright and
                      I. Thiel and S. Bordbari and M. Domnich and E. Siakaeva and
                      A. Lakomek and F. Toppe and C. Schleupner and U. Geisthoff
                      and S. Lang$^*$ and F. Droege and J. Jablonska$^*$},
      title        = {{N}on-functional {TGF}-β/{ALK}1/{ENG} {S}ignaling
                      {P}athway supports neutrophil pro-angiogenic activity in
                      {H}ereditary {H}emorrhagic {T}elangiectasia},
      journal      = {Journal of leukocyte biology},
      volume       = {114},
      number       = {6},
      issn         = {0741-5400},
      address      = {Hoboken, NJ},
      publisher    = {Wiley},
      reportid     = {DKFZ-2023-01619},
      pages        = {639-650},
      year         = {2023},
      note         = {2023 Nov 24;114(6):639-650},
      abstract     = {TGF-β/ALK1/ENG signaling pathway maintains quiescent state
                      of endothelial cells, but at the same time, it regulates
                      neutrophil functions. Importantly, mutations of this pathway
                      lead to a rare autosomal disorder called Hereditary
                      Hemorrhagic Telangiectasia (HHT), characterized with
                      abnormal blood vessel formation (angiogenesis). As
                      neutrophils are potent regulators of angiogenesis, we
                      investigated how disturbed TGF-β/ALK1/ENG signaling
                      influences angiogenic properties of these cells in HHT. We
                      could show for the first time that not only endothelial
                      cells, but also neutrophils isolated from such patients are
                      ENG/ALK1-deficient. This deficiency obviously stimulates
                      proangiogenic switch of such neutrophils. Elevated
                      proangiogenic activity of HHT neutrophils is mediated by the
                      increased spontaneous degranulation of gelatinase granules,
                      resulting in high release of matrix-degrading MMP9. In
                      agreement, therapeutic disturbance of this process using Src
                      tyrosine kinase inhibitors impaired proangiogenic capacity
                      of such neutrophils. Similarly, inhibition of MMP9 activity
                      resulted in significant impairment of neutrophil-mediated
                      angiogenesis. All in all, deficiency in TGF-β/ALK1/ENG
                      signaling in HHT neutrophils results in their proangiogenic
                      activation and disease progression. Therapeutic strategies
                      targeting neutrophil degranulation and MMP9 release and
                      activity may serve as a potential therapeutic option for
                      HHT.},
      cin          = {ED01},
      ddc          = {570},
      cid          = {I:(DE-He78)ED01-20160331},
      pnm          = {899 - ohne Topic (POF4-899)},
      pid          = {G:(DE-HGF)POF4-899},
      typ          = {PUB:(DE-HGF)16},
      doi          = {10.1093/jleuko/qiad090},
      url          = {https://inrepo02.dkfz.de/record/278369},
}