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@ARTICLE{Hansen:164661,
      author       = {Hansen, Jan N and Kaiser, Fabian and Leyendecker, Philipp
                      and Stüven, Birthe and Krause, Jens-Henning and
                      Derakhshandeh, Fatemeh and Irfan, Jaazba and Sroka, Tommy J
                      and Preval, Kenley M and Desai, Paurav B and Kraut, Michael
                      and Theis, Heidi and Drews, Anna-Dorothee and de Domenico,
                      Elena and Händler, Kristian and Pazour, Gregory J and
                      Henderson, David J P and Mick, David U and Wachten, Dagmar},
      title        = {{A} c{AMP} signalosome in primary cilia drives gene
                      expression and kidney cyst formation},
      journal      = {EMBO reports},
      volume       = {23},
      number       = {8},
      issn         = {1469-221X},
      address      = {Hoboken, NJ [u.a.]},
      publisher    = {Wiley},
      reportid     = {DZNE-2022-01191},
      pages        = {e54315},
      year         = {2022},
      note         = {(CC BY-NC-ND)},
      abstract     = {The primary cilium constitutes an organelle that
                      orchestrates signal transduction independently from the cell
                      body. Dysregulation of this intricate molecular architecture
                      leads to severe human diseases, commonly referred to as
                      ciliopathies. However, the molecular underpinnings how
                      ciliary signaling orchestrates a specific cellular output
                      remain elusive. By combining spatially resolved optogenetics
                      with RNA sequencing and imaging, we reveal a novel cAMP
                      signalosome that is functionally distinct from the
                      cytoplasm. We identify the genes and pathways targeted by
                      the ciliary cAMP signalosome and shed light on the
                      underlying mechanisms and downstream signaling. We reveal
                      that chronic stimulation of the ciliary cAMP signalosome
                      transforms kidney epithelia from tubules into cysts.
                      Counteracting this chronic cAMP elevation in the cilium by
                      small molecules targeting activation of phosphodiesterase-4
                      long isoforms inhibits cyst growth. Thereby, we identify a
                      novel concept of how the primary cilium controls cellular
                      functions and maintains tissue integrity in a specific and
                      spatially distinct manner and reveal novel molecular
                      components that might be involved in the development of one
                      of the most common genetic diseases, polycystic kidney
                      disease.},
      keywords     = {Cilia: metabolism / Cysts: metabolism / Gene Expression /
                      Humans / Kidney / Polycystic Kidney Diseases: genetics /
                      Polycystic Kidney Diseases: metabolism / CREB (Other) / PKD
                      (Other) / cAMP (Other) / optogenetics (Other) / primary
                      cilia (Other)},
      cin          = {AG Schultze / $R\&D$ PRECISE},
      ddc          = {570},
      cid          = {I:(DE-2719)1013031 / I:(DE-2719)5000031},
      pnm          = {354 - Disease Prevention and Healthy Aging (POF4-354)},
      pid          = {G:(DE-HGF)POF4-354},
      typ          = {PUB:(DE-HGF)16},
      pmc          = {pmc:PMC9346484},
      pubmed       = {pmid:35695071},
      doi          = {10.15252/embr.202154315},
      url          = {https://pub.dzne.de/record/164661},
}