{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T08:08:03Z","timestamp":1772784483823,"version":"3.50.1"},"reference-count":67,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2021,11,3]],"date-time":"2021-11-03T00:00:00Z","timestamp":1635897600000},"content-version":"vor","delay-in-days":601,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2020,3,12]],"date-time":"2020-03-12T00:00:00Z","timestamp":1583971200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2020,3,12]],"date-time":"2020-03-12T00:00:00Z","timestamp":1583971200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100002997","name":"Nierstichting","doi-asserted-by":"publisher","award":["KBAO1205"],"award-info":[{"award-number":["KBAO1205"]}],"id":[{"id":"10.13039\/501100002997","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003246","name":"Netherlands Organisation for Scientific Research","doi-asserted-by":"publisher","award":["NWO 022.004.002"],"award-info":[{"award-number":["NWO 022.004.002"]}],"id":[{"id":"10.13039\/501100003246","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002997","name":"Nierstichting","doi-asserted-by":"publisher","award":["KBAO1205"],"award-info":[{"award-number":["KBAO1205"]}],"id":[{"id":"10.13039\/501100002997","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003246","name":"Nederlandse Organisatie voor Wetenschappelijk Onderzoek","doi-asserted-by":"publisher","award":["NWO 022.004.002"],"award-info":[{"award-number":["NWO 022.004.002"]}],"id":[{"id":"10.13039\/501100003246","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2020,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Autosomal-dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease, leading to kidney failure in most patients. In approximately 85% of cases, the disease is caused by mutations in PKD1. How dysregulation of PKD1 leads to cyst formation on a molecular level is unknown. Induced pluripotent stem cells (iPSCs) are a powerful tool for in vitro modeling of genetic disorders. Here, we established ADPKD patient-specific iPSCs to study the function of PKD1 in kidney development and cyst formation in vitro. Somatic mutations are proposed to be the initiating event of cyst formation, and therefore, iPSCs were derived from cystic renal epithelial cells rather than fibroblasts. Mutation analysis of the ADPKD iPSCs revealed germline mutations in PKD1 but no additional somatic mutations in PKD1\/PKD2. Although several somatic mutations in other genes implicated in ADPKD were identified in cystic renal epithelial cells, only few of these mutations were present in iPSCs, indicating a heterogeneous mutational landscape, and possibly in vitro cell selection before and during the reprogramming process. Whole-genome DNA methylation analysis indicated that iPSCs derived from renal epithelial cells maintain a kidney-specific DNA methylation memory. In addition, comparison of PKD1+\/\u2212 and control iPSCs revealed differences in DNA methylation associated with the disease history. In conclusion, we generated and characterized iPSCs derived from cystic and healthy control renal epithelial cells, which can be used for in vitro modeling of kidney development in general and cystogenesis in particular.<\/jats:p>\n               <jats:sec>\n                  <jats:title\/>\n                  <jats:p>Significance statement Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease, leading to kidney failure in most patients. In approximately 85% of cases, the disease is caused by mutations in PKD1. How dysregulation of PKD1 leads to cyst formation on a molecular level is unknown. The present study has generated induced pluripotent stem cells (iPSCs) of ADPKD patients to study the function of PKD1 in kidney development and cyst formation in vitro. The iPSCs revealed germline and autosomal mutations implicated in ADPKD and displayed an epigenetic memory of kidney epithelial cells, providing powerful models to study ADPKD in vitro.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1002\/sctm.18-0283","type":"journal-article","created":{"date-parts":[[2020,3,12]],"date-time":"2020-03-12T12:48:06Z","timestamp":1584017286000},"page":"478-490","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Cystic renal-epithelial derived induced pluripotent stem cells from polycystic kidney disease patients"],"prefix":"10.1093","volume":"9","author":[{"given":"Annegien T.","family":"Kenter","sequence":"first","affiliation":[{"name":"1 Department of Developmental Biology Erasmus Medical Center Rotterdam (EMC), Oncode Institute, Rotterdam, The Netherlands"},{"name":"2 Department of Cell Biology Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"},{"name":"3 Department of Internal Medicine, Division of Nephrology and Transplantation Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eveline","family":"Rentmeester","sequence":"additional","affiliation":[{"name":"1 Department of Developmental Biology Erasmus Medical Center Rotterdam (EMC), Oncode Institute, Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Job","family":"Riet","sequence":"additional","affiliation":[{"name":"4 Cancer Computational Biology Center Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ruben","family":"Boers","sequence":"additional","affiliation":[{"name":"1 Department of Developmental Biology Erasmus Medical Center Rotterdam (EMC), Oncode Institute, Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joachim","family":"Boers","sequence":"additional","affiliation":[{"name":"1 Department of Developmental Biology Erasmus Medical Center Rotterdam (EMC), Oncode Institute, Rotterdam, The Netherlands"},{"name":"5 Delft Diagnostic Laboratories (DDL), Rijswijk, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mehrnaz","family":"Ghazvini","sequence":"additional","affiliation":[{"name":"1 Department of Developmental Biology Erasmus Medical Center Rotterdam (EMC), Oncode Institute, Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vanessa J.","family":"Xavier","sequence":"additional","affiliation":[{"name":"1 Department of Developmental Biology Erasmus Medical Center Rotterdam (EMC), Oncode Institute, Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Geert J.L.H.","family":"Leenders","sequence":"additional","affiliation":[{"name":"6 Department of Pathology Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul C.M.S.","family":"Verhagen","sequence":"additional","affiliation":[{"name":"7 Department of Urology Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marjan E.","family":"Til","sequence":"additional","affiliation":[{"name":"8 Department of Clinical Genetics Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bert","family":"Eussen","sequence":"additional","affiliation":[{"name":"8 Department of Clinical Genetics Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Monique","family":"Losekoot","sequence":"additional","affiliation":[{"name":"9 Department of Clinical Genetics Leiden University Medical Center (LUMC), Leiden, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annelies","family":"Klein","sequence":"additional","affiliation":[{"name":"8 Department of Clinical Genetics Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dorien J.M.","family":"Peters","sequence":"additional","affiliation":[{"name":"10 Department of Human Genetics Leiden University Medical Center (LUMC), Leiden, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wilfred F.J.","family":"IJcken","sequence":"additional","affiliation":[{"name":"11 Erasmus Center for Biomics Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Harmen J.G.","family":"Werken","sequence":"additional","affiliation":[{"name":"4 Cancer Computational Biology Center Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"Zietse","sequence":"additional","affiliation":[{"name":"3 Department of Internal Medicine, Division of Nephrology and Transplantation Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ewout J.","family":"Hoorn","sequence":"additional","affiliation":[{"name":"3 Department of Internal Medicine, Division of Nephrology and Transplantation Erasmus Medical Center Rotterdam (EMC), Rotterdam, The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gert","family":"Jansen","sequence":"additional","affiliation":[{"name":"2 Department of Cell Biology 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