{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T20:29:30Z","timestamp":1790972970011,"version":"4.1.0"},"reference-count":60,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"10","funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["JP17H06177"],"award-info":[{"award-number":["JP17H06177"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["20bm0804013h0004"],"award-info":[{"award-number":["20bm0804013h0004"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JASN"],"published-print":{"date-parts":[[2020,10]]},"abstract":"\n Significance Statement<\/jats:title>\n \n Models of autosomal dominant polycystic kidney disease (ADPKD) are possible in nephron progenitor\u2013derived human kidney organoids that form from induced pluripotent stem cells (iPSCs) that have had\n PKD<\/jats:italic>\n genes deleted, enabling cyst formation. However, this has not been achieved in the ureteric bud\/collecting duct lineage, despite the prevalence of collecting duct cysts in patients. Cysts formed in ureteric bud organoids derived from iPSCs with homozygous deleted\n PKD1<\/jats:italic>\n , as well as in ureteric bud organoids generated from heterozygous mutant iPSCs and from a patient with ADPKD who had a heterozygous missense mutation, all upon cAMP stimulation. These\n PKD1<\/jats:italic>\n mutant organoids can model human ADPKD in the collecting duct lineage, complementing existing cell and animal models.\n <\/jats:p>\n <\/jats:sec>\n \n Background<\/jats:title>\n \n Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease leading to renal failure, wherein multiple cysts form in renal tubules and collecting ducts derived from distinct precursors: the nephron progenitor and ureteric bud (UB), respectively. Recent progress in induced pluripotent stem cell (iPSC) biology has enabled cyst formation in nephron progenitor\u2013derived human kidney organoids in which\n PKD1<\/jats:italic>\n or\n PKD2<\/jats:italic>\n , the major causative genes for ADPKD, are deleted. However, cysts have not been generated in UB organoids, despite the prevalence of collecting duct cysts in patients with ADPKD.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n CRISPR-Cas9 technology deleted\n PKD1<\/jats:italic>\n in human iPSCs and the cells induced to differentiate along pathways leading to formation of either nephron progenitor or UB organoids. Cyst formation was investigated in both types of kidney organoid derived from\n PKD1<\/jats:italic>\n -deleted iPSCs and in UB organoids generated from iPSCs from a patient with ADPKD who had a missense mutation.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Cysts formed in UB organoids with homozygous\n PKD1<\/jats:italic>\n mutations upon cAMP stimulation and, to a lesser extent, in heterozygous mutant organoids. Furthermore, UB organoids generated from iPSCs from a patient with ADPKD who had a heterozygous missense mutation developed cysts upon cAMP stimulation.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n Cysts form in\n PKD1<\/jats:italic>\n mutant UB organoids as well as in iPSCs derived from a patient with ADPKD. The organoids provide a robust model of the genesis of ADPKD.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1681\/asn.2020030378","type":"journal-article","created":{"date-parts":[[2020,8,3]],"date-time":"2020-08-03T16:54:55Z","timestamp":1596473695000},"page":"2355-2371","source":"Crossref","is-referenced-by-count":92,"title":["PKD1-Dependent Renal Cystogenesis in Human Induced Pluripotent Stem Cell-Derived Ureteric Bud\/Collecting Duct Organoids"],"prefix":"10.1681","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1599-5162","authenticated-orcid":false,"given":"Shohei","family":"Kuraoka","sequence":"first","affiliation":[{"name":"Department of Kidney Development, Institute of Molecular Embryology and Genetics, Kumamoto University, Kumamoto, Japan"},{"name":"Department of Pediatrics, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shunsuke","family":"Tanigawa","sequence":"additional","affiliation":[{"name":"Department of Kidney Development, Institute of Molecular Embryology and Genetics, Kumamoto University, Kumamoto, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsuhiro","family":"Taguchi","sequence":"additional","affiliation":[{"name":"Department of Kidney Development, Institute of Molecular Embryology and Genetics, Kumamoto University, Kumamoto, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akitsu","family":"Hotta","sequence":"additional","affiliation":[{"name":"Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University, Kyoto, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hitoshi","family":"Nakazato","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenji","family":"Osafune","sequence":"additional","affiliation":[{"name":"Department of Cell Growth and Differentiation, Center for iPS Cell Research and Application, Kyoto University, Kyoto, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akio","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Department of Kidney Development, Institute of Molecular Embryology and Genetics, Kumamoto University, Kumamoto, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryuichi","family":"Nishinakamura","sequence":"additional","affiliation":[{"name":"Department of Kidney Development, Institute of Molecular Embryology and Genetics, Kumamoto University, Kumamoto, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2020,8,3]]},"reference":[{"key":"B1-20230829","doi-asserted-by":"crossref","first-page":"2069","DOI":"10.1093\/ndt\/12.10.2069","article-title":"Frequency and impact of autosomal dominant polycystic kidney disease in the Seychelles (Indian Ocean)","volume":"12","author":"Yersin","year":"1997","journal-title":"Nephrol Dial Transplant"},{"key":"B2-20230829","doi-asserted-by":"crossref","first-page":"1287","DOI":"10.1016\/S0140-6736(07)60601-1","article-title":"Autosomal dominant polycystic kidney disease","volume":"369","author":"Torres","year":"2007","journal-title":"Lancet"},{"key":"B3-20230829","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1172\/JCI21753","article-title":"Mechanical stimuli induce cleavage and nuclear translocation of the polycystin-1 C terminus [published correction appears in J Clin Invest 115: 788, 2005]","volume":"114","author":"Chauvet","year":"2004","journal-title":"J Clin Invest"},{"key":"B4-20230829","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1038\/ng1076","article-title":"Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells","volume":"33","author":"Nauli","year":"2003","journal-title":"Nat Genet"},{"key":"B5-20230829","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1038\/nrneph.2010.97","article-title":"Polycystins and renovascular mechanosensory transduction","volume":"6","author":"Patel","year":"2010","journal-title":"Nat Rev Nephrol"},{"key":"B6-20230829","doi-asserted-by":"crossref","first-page":"752","DOI":"10.1038\/ncb3363","article-title":"The polycystin complex mediates Wnt\/Ca(2+) signalling","volume":"18","author":"Kim","year":"2016","journal-title":"Nat Cell Biol"},{"key":"B7-20230829","doi-asserted-by":"crossref","first-page":"990","DOI":"10.1038\/35050128","article-title":"Co-assembly of polycystin-1 and -2 produces unique cation-permeable currents","volume":"408","author":"Hanaoka","year":"2000","journal-title":"Nature"},{"key":"B8-20230829","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1006\/bbrc.2001.4554","article-title":"Polycystin-2 is a novel cation channel implicated in defective intracellular Ca(2+) homeostasis in polycystic kidney disease","volume":"282","author":"Vassilev","year":"2001","journal-title":"Biochem Biophys Res Commun"},{"key":"B9-20230829","doi-asserted-by":"crossref","first-page":"eaat9819","DOI":"10.1126\/science.aat9819","article-title":"Structure of the human PKD1-PKD2 complex","volume":"361","author":"Su","year":"2018","journal-title":"Science"},{"key":"B10-20230829","doi-asserted-by":"crossref","first-page":"814","DOI":"10.1681\/ASN.V115814","article-title":"Cellular and subcellular distribution of polycystin-2, the protein product of the PKD2 gene","volume":"11","author":"Foggensteiner","year":"2000","journal-title":"J Am Soc Nephrol"},{"key":"B11-20230829","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1146\/annurev.physiol.70.113006.100621","article-title":"Polycystins and primary cilia: Primers for cell cycle progression","volume":"71","author":"Zhou","year":"2009","journal-title":"Annu Rev Physiol"},{"key":"B12-20230829","doi-asserted-by":"crossref","first-page":"656","DOI":"10.1038\/nature17426","article-title":"Primary cilia are not calcium-responsive mechanosensors","volume":"531","author":"Delling","year":"2016","journal-title":"Nature"},{"key":"B13-20230829","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1016\/S1471-4914(01)01953-0","article-title":"A \u201ctwo-hit\u201d model of cystogenesis in autosomal dominant polycystic kidney disease?","volume":"7","author":"Pei","year":"2001","journal-title":"Trends Mol Med"},{"key":"B14-20230829","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1007\/s00467-006-0327-8","article-title":"Prognosis of autosomal dominant polycystic kidney disease diagnosed in utero or at birth","volume":"22","author":"Boyer","year":"2007","journal-title":"Pediatr Nephrol"},{"key":"B15-20230829","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1053\/j.ajkd.2010.02.349","article-title":"Renal ultrasonographic evaluation in children at risk of autosomal dominant polycystic kidney disease","volume":"56","author":"Reed","year":"2010","journal-title":"Am J Kidney Dis"},{"key":"B16-20230829","doi-asserted-by":"crossref","first-page":"889","DOI":"10.2215\/CJN.00550110","article-title":"Evidence of extraordinary growth in the progressive enlargement of renal cysts","volume":"5","author":"Grantham","year":"2010","journal-title":"Clin J Am Soc Nephrol"},{"key":"B17-20230829","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1038\/ng1097-179","article-title":"Perinatal lethality with kidney and pancreas defects in mice with a targetted Pkd1 mutation","volume":"17","author":"Lu","year":"1997","journal-title":"Nat Genet"},{"key":"B18-20230829","doi-asserted-by":"crossref","first-page":"2385","DOI":"10.1093\/hmg\/10.21.2385","article-title":"Comparison of Pkd1-targeted mutants reveals that loss of polycystin-1 causes cystogenesis and bone defects","volume":"10","author":"Lu","year":"2001","journal-title":"Hum Mol Genet"},{"key":"B19-20230829","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1038\/5944","article-title":"Late onset of renal and hepatic cysts in Pkd1-targeted heterozygotes","volume":"21","author":"Lu","year":"1999","journal-title":"Nat Genet"},{"key":"B20-20230829","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.stem.2013.11.010","article-title":"Redefining the in vivo origin of metanephric nephron progenitors enables generation of complex kidney structures from pluripotent stem cells","volume":"14","author":"Taguchi","year":"2014","journal-title":"Cell Stem Cell"},{"key":"B21-20230829","doi-asserted-by":"crossref","first-page":"1193","DOI":"10.1038\/nbt.3392","article-title":"Nephron organoids derived from human pluripotent stem cells model kidney development and injury","volume":"33","author":"Morizane","year":"2015","journal-title":"Nat Biotechnol"},{"key":"B22-20230829","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1016\/j.stem.2017.10.011","article-title":"Higher-order kidney organogenesis from pluripotent stem cells","volume":"21","author":"Taguchi","year":"2017","journal-title":"Cell Stem Cell"},{"key":"B23-20230829","doi-asserted-by":"crossref","first-page":"954","DOI":"10.1016\/j.bbrc.2017.11.105","article-title":"Generation of branching ureteric bud tissues from human pluripotent stem cells","volume":"495","author":"Mae","year":"2018","journal-title":"Biochem Biophys Res Commun"},{"key":"B24-20230829","doi-asserted-by":"crossref","first-page":"564","DOI":"10.1038\/nature15695","article-title":"Kidney organoids from human iPS cells contain multiple lineages and model human nephrogenesis","volume":"526","author":"Takasato","year":"2015","journal-title":"Nature"},{"key":"B25-20230829","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1126\/science.1225829","article-title":"A programmable dual-RNA-guided DNA endonuclease in adaptive bacterial immunity","volume":"337","author":"Jinek","year":"2012","journal-title":"Science"},{"key":"B26-20230829","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.ymeth.2015.10.015","article-title":"Efficient genomic correction methods in human iPS cells using CRISPR-Cas9 system","volume":"101","author":"Li","year":"2016","journal-title":"Methods"},{"key":"B27-20230829","doi-asserted-by":"crossref","first-page":"8715","DOI":"10.1038\/ncomms9715","article-title":"Modelling kidney disease with CRISPR-mutant kidney organoids derived from human pluripotent epiblast spheroids","volume":"6","author":"Freedman","year":"2015","journal-title":"Nat Commun"},{"key":"B28-20230829","doi-asserted-by":"crossref","first-page":"1112","DOI":"10.1038\/nmat4994","article-title":"Organoid cystogenesis reveals a critical role of microenvironment in human polycystic kidney disease","volume":"16","author":"Cruz","year":"2017","journal-title":"Nat Mater"},{"key":"B29-20230829","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1016\/j.stem.2018.04.022","article-title":"High-throughput screening enhances kidney organoid differentiation from human pluripotent stem cells and enables automated multidimensional phenotyping","volume":"22","author":"Czerniecki","year":"2018","journal-title":"Cell Stem Cell"},{"key":"B30-20230829","first-page":"F169","article-title":"Expression of aquaporins-1 and -2 during nephrogenesis and in autosomal dominant polycystic kidney disease","volume":"271","author":"Devuyst","year":"1996","journal-title":"Am J Physiol"},{"key":"B31-20230829","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1002\/phar.1421","article-title":"Review of tolvaptan for autosomal dominant polycystic kidney disease","volume":"34","author":"Baur","year":"2014","journal-title":"Pharmacotherapy"},{"key":"B32-20230829","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1016\/j.stem.2018.10.010","article-title":"Comparative analysis and refinement of human PSC-derived kidney organoid differentiation with single-cell transcriptomics","volume":"23","author":"Wu","year":"2018","journal-title":"Cell Stem Cell"},{"key":"B33-20230829","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1186\/s13073-019-0615-0","article-title":"Single-cell analysis reveals congruence between kidney organoids and human fetal kidney","volume":"11","author":"Combes","year":"2019","journal-title":"Genome Med"},{"key":"B34-20230829","doi-asserted-by":"crossref","first-page":"1778","DOI":"10.1681\/ASN.2015010096","article-title":"Human induced pluripotent stem cell-derived podocytes mature into vascularized glomeruli upon experimental transplantation","volume":"27","author":"Sharmin","year":"2016","journal-title":"J Am Soc Nephrol"},{"key":"B35-20230829","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1681\/ASN.2018070747","article-title":"Manipulation of nephron-patterning signals enables selective induction of podocytes from human pluripotent stem cells","volume":"30","author":"Yoshimura","year":"2019","journal-title":"J Am Soc Nephrol"},{"key":"B36-20230829","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1538\/expanim.63.79","article-title":"Screening methods to identify TALEN-mediated knockout mice","volume":"63","author":"Nakagawa","year":"2014","journal-title":"Exp Anim"},{"key":"B37-20230829","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1038\/sj.embor.7400787","article-title":"Structural basis for the fast maturation of Arthropoda green fluorescent protein","volume":"7","author":"Evdokimov","year":"2006","journal-title":"EMBO Rep"},{"key":"B38-20230829","doi-asserted-by":"crossref","first-page":"861","DOI":"10.1016\/j.cell.2007.11.019","article-title":"Induction of pluripotent stem cells from adult human fibroblasts by defined factors","volume":"131","author":"Takahashi","year":"2007","journal-title":"Cell"},{"key":"B39-20230829","doi-asserted-by":"crossref","first-page":"3745","DOI":"10.1093\/nar\/25.18.3745","article-title":"A transgenic mouse strain expressing four drug-selectable marker genes","volume":"25","author":"Tucker","year":"1997","journal-title":"Nucleic Acids Res"},{"key":"B41-20230829","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1016\/j.stem.2016.07.016","article-title":"3D culture supports long-term expansion of mouse and human nephrogenic progenitors","volume":"19","author":"Li","year":"2016","journal-title":"Cell Stem Cell"},{"key":"B42-20230829","doi-asserted-by":"crossref","first-page":"30013","DOI":"10.1038\/srep30013","article-title":"Identification of MMP1 as a novel risk factor for intracranial aneurysms in ADPKD using iPSC models","volume":"6","author":"Ameku","year":"2016","journal-title":"Sci Rep"},{"key":"B43-20230829","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1038\/nmeth.2089","article-title":"NIH image to ImageJ: 25 years of image analysis","volume":"9","author":"Schneider","year":"2012","journal-title":"Nat Methods"},{"key":"B44-20230829","doi-asserted-by":"crossref","first-page":"1044","DOI":"10.1086\/301600","article-title":"Mutation detection in the repeated part of the PKD1 gene","volume":"61","author":"Roelfsema","year":"1997","journal-title":"Am J Hum Genet"},{"key":"B45-20230829","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1186\/1471-2148-8-263","article-title":"Chromosomal evolution of the PKD1 gene family in primates [published correction appears in BMC Evol Biol 9: 14, 2009]","volume":"8","author":"Kirsch","year":"2008","journal-title":"BMC Evol Biol"},{"key":"B46-20230829","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.1681\/ASN.2015010047","article-title":"Phosphodiesterase isoform regulation of cell proliferation and fluid secretion in autosomal dominant polycystic kidney disease","volume":"27","author":"Pinto","year":"2016","journal-title":"J Am Soc Nephrol"},{"key":"B47-20230829","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.1093\/ndt\/gfp611","article-title":"Glomerular and proximal tubule cysts as early manifestations of Pkd1 deletion","volume":"25","author":"Ahrabi","year":"2010","journal-title":"Nephrol Dial Transplant"},{"key":"B48-20230829","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1038\/ng.400","article-title":"Wnt9b signaling regulates planar cell polarity and kidney tubule morphogenesis","volume":"41","author":"Karner","year":"2009","journal-title":"Nat Genet"},{"key":"B49-20230829","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1067\/mlc.2003.13","article-title":"Autosomal dominant polycystic kidney disease: Molecular genetics and pathophysiology","volume":"141","author":"Sutters","year":"2003","journal-title":"J Lab Clin Med"},{"key":"B50-20230829","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1053\/j.ajkd.2012.08.038","article-title":"Relationship of copeptin, a surrogate marker for arginine vasopressin, with change in total kidney volume and GFR decline in autosomal dominant polycystic kidney disease: Results from the CRISP cohort","volume":"61","author":"Boertien","year":"2013","journal-title":"Am J Kidney Dis"},{"key":"B51-20230829","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1186\/s13059-015-0690-5","article-title":"Statistically based splicing detection reveals neural enrichment and tissue-specific induction of circular RNA during human fetal development [published correction appears in Genome Biol 17: 263, 2016]","volume":"16","author":"Szabo","year":"2015","journal-title":"Genome Biol"},{"key":"B52-20230829","doi-asserted-by":"crossref","first-page":"2872","DOI":"10.1681\/ASN.2015050583","article-title":"Predicted mutation strength of nontruncating PKD1 mutations aids genotype-phenotype correlations in autosomal dominant polycystic kidney disease","volume":"27","author":"Heyer","year":"2016","journal-title":"J Am Soc Nephrol"},{"key":"B53-20230829","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1093\/hmg\/5.4.539","article-title":"A stable, nonsense mutation associated with a case of infantile onset polycystic kidney disease 1 (PKD1)","volume":"5","author":"Peral","year":"1996","journal-title":"Hum Mol Genet"},{"key":"B54-20230829","doi-asserted-by":"crossref","first-page":"5704","DOI":"10.1038\/s41598-018-23732-5","article-title":"Activation of calcium-sensing receptor increases intracellular calcium and decreases cAMP and mTOR in PKD1 deficient cells","volume":"8","author":"Di Mise","year":"2018","journal-title":"Sci Rep"},{"key":"B55-20230829","doi-asserted-by":"crossref","first-page":"964","DOI":"10.1111\/j.1523-1755.2004.00843.x","article-title":"Cyclic AMP promotes growth and secretion in human polycystic kidney epithelial cells","volume":"66","author":"Belibi","year":"2004","journal-title":"Kidney Int"},{"key":"B56-20230829","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1097\/00000542-200609000-00026","article-title":"The vasopressin system: Physiology and clinical strategies","volume":"105","author":"Treschan","year":"2006","journal-title":"Anesthesiology"},{"key":"B57-20230829","doi-asserted-by":"crossref","first-page":"8772","DOI":"10.1074\/jbc.271.15.8772","article-title":"Different single receptor domains determine the distinct G protein coupling profiles of members of the vasopressin receptor family","volume":"271","author":"Liu","year":"1996","journal-title":"J Biol Chem"},{"key":"B58-20230829","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1016\/j.devcel.2019.10.005","article-title":"Single-cell profiling reveals sex, lineage, and regional diversity in the mouse kidney","volume":"51","author":"Ransick","year":"2019","journal-title":"Dev Cell"},{"key":"B59-20230829","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1242\/dmm.003194","article-title":"The ADPKD genes pkd1a\/b and pkd2 regulate extracellular matrix formation","volume":"3","author":"Mangos","year":"2010","journal-title":"Dis Model Mech"},{"key":"B60-20230829","doi-asserted-by":"crossref","first-page":"5517","DOI":"10.1038\/s41467-019-13398-6","article-title":"Monkeys mutant for PKD1 recapitulate human autosomal dominant polycystic kidney disease","volume":"10","author":"Tsukiyama","year":"2019","journal-title":"Nat Commun"},{"key":"B61-20230829","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1038\/s41592-019-0325-y","article-title":"Flow-enhanced vascularization and maturation of kidney organoids in vitro","volume":"16","author":"Homan","year":"2019","journal-title":"Nat Methods"}],"container-title":["Journal of the American Society of Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.1681\/ASN.2020030378","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T19:24:16Z","timestamp":1786130656000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.1681\/ASN.2020030378"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8,3]]},"references-count":60,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2020]]}},"URL":"https:\/\/doi.org\/10.1681\/asn.2020030378","relation":{},"ISSN":["1046-6673","1533-3450"],"issn-type":[{"value":"1046-6673","type":"print"},{"value":"1533-3450","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,8,3]]}}}