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Here, we show that a C\u2010terminal fragment of human FC can suppress a signalling module of the kinase SRC and signal transducer and activator of transcription 3 (STAT3). Consistently, we identified truncating genetic variants specifically affecting the cytoplasmic tail in ARPKD patients, found SRC and the cytoplasmic tail of fibrocystin in a joint dynamic protein complex and observed increased activation of both SRC and STAT3 in cyst\u2010lining renal epithelial cells of ARPKD patients.\n                  <\/jats:p>","DOI":"10.1111\/jcmm.16014","type":"journal-article","created":{"date-parts":[[2020,10,28]],"date-time":"2020-10-28T05:53:25Z","timestamp":1603864405000},"page":"14633-14638","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["The carboxy\u2010terminus of the human ARPKD protein fibrocystin can control STAT3 signalling by regulating SRC\u2010activation"],"prefix":"10.1111","volume":"24","author":[{"given":"Claudia","family":"Dafinger","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"Department II of Internal Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"Center for Molecular Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amrei M.","family":"Mandel","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"Department II of Internal Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alina","family":"Braun","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"Department II of Internal Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heike","family":"G\u00f6bel","sequence":"additional","affiliation":[{"name":"Institute of Pathology Faculty of Medicine University Hospital Cologne and University of Cologne Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathrin","family":"Burgmaier","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Massella","sequence":"additional","affiliation":[{"name":"Nephrology and Dialysis Unit Bambino Ges\u00f9 Children's Hospital IRCCS Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Mastrangelo","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology, Dialysis and Transplant Unit Fondazione IRCCS C\u00e0 Granda Ospedale Maggiore Policlinico Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rg","family":"D\u00f6tsch","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Benzing","sequence":"additional","affiliation":[{"name":"Department II of Internal Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"Center for Molecular Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"CECAD University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"Systems Biology of Ageing Cologne University of Cologne Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Weimbs","sequence":"additional","affiliation":[{"name":"Molecular, Cellular, and Developmental Biology, and Neuroscience Research Institute University of California Santa Barbara CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernhard","family":"Schermer","sequence":"additional","affiliation":[{"name":"Department II of Internal Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"Center for Molecular Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"CECAD University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"Systems Biology of Ageing Cologne University of Cologne Cologne Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0494-9080","authenticated-orcid":false,"given":"Max C.","family":"Liebau","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"Department II of Internal Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne Germany"},{"name":"Center for Molecular Medicine University of Cologne Faculty of Medicine and University Hospital Cologne Cologne 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