{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T16:55:08Z","timestamp":1772384108514,"version":"3.50.1"},"reference-count":18,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2010,10,11]],"date-time":"2010-10-11T00:00:00Z","timestamp":1286755200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["pathsocjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Pathology"],"published-print":{"date-parts":[[2011,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Autosomal dominant polycystic kidney disease (PKD) is an inherited disease that results from mutations in either polycystin (PKD1) or polycystin 2 (PKD2), both of which are large, complex, and multifunctional proteins whose loss results in the development of numerous fluid\u2010filled cysts and fibrosis that compromise renal function. A number of spontaneous and engineered mouse models of PKD have provided some understanding of many aspects of cyst development, modifier genes, and mechanistic pathways, but fall short of reproducing the human disease accurately. Two recent papers in <jats:italic>The Journal of Pathology<\/jats:italic> set out new models using miRNA, or inducible and targeted recombination, that achieve partial or timed suppression of <jats:italic>Pkd1.<\/jats:italic> Instead of knocking out <jats:italic>Pkd1<\/jats:italic> immediately, or completely, these more subtle approaches may help deliver more faithful models of this significant human renal disease. Copyright \u00a9 2010 Pathological Society of Great Britain and Ireland. Published by John Wiley &amp; Sons, Ltd.<\/jats:p><jats:p>Invited Commentary for Hassane S, Leonhard WN, van der Wal A, <jats:italic>et al.<\/jats:italic> Elevated TGF\u03b2\u2013Smad signalling in experimental <jats:italic>Pkd1<\/jats:italic> models and human patients with polycystic kidney disease. <jats:italic>J Pathol<\/jats:italic> 2010; <jats:bold>222:<\/jats:bold> 21\u201331. And for Wang E, Hsieh\u2010Li HM, Chiou YY, <jats:italic>et al.<\/jats:italic> Progressive renal distortion by multiple cysts in transgenic mice expressing artificial microRNAs against <jats:italic>Pkd1. J Pathol<\/jats:italic> 2010; <jats:bold>222<\/jats:bold>: 238\u2013248.<\/jats:p>","DOI":"10.1002\/path.2782","type":"journal-article","created":{"date-parts":[[2010,9,27]],"date-time":"2010-09-27T03:59:22Z","timestamp":1285559962000},"page":"1-3","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["In the ring with polycystic kidney disease\u2014avoiding the knockout punch"],"prefix":"10.1002","volume":"223","author":[{"given":"Julie","family":"Adam","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrick J","family":"Pollard","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2010,10,11]]},"reference":[{"key":"e_1_2_3_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra022161"},{"key":"e_1_2_3_3_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.med.60.101707.125712"},{"key":"e_1_2_3_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1523-1755.2005.00201.x"},{"key":"e_1_2_3_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81793-6"},{"key":"e_1_2_3_6_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddh336"},{"key":"e_1_2_3_7_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddm299"},{"key":"e_1_2_3_8_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2796.2006.01743.x"},{"key":"e_1_2_3_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/path.2734"},{"key":"e_1_2_3_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/path.2765"},{"key":"e_1_2_3_11_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng1097-179"},{"key":"e_1_2_3_12_2","doi-asserted-by":"publisher","DOI":"10.1038\/71724"},{"key":"e_1_2_3_13_2","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2006.050342"},{"key":"e_1_2_3_14_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.ASN.0000144204.01352.86"},{"key":"e_1_2_3_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2008.09.061"},{"key":"e_1_2_3_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(03)00432-X"},{"key":"e_1_2_3_17_2","doi-asserted-by":"publisher","DOI":"10.1038\/nm.1865"},{"key":"e_1_2_3_18_2","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-05-3241"},{"key":"e_1_2_3_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccr.2007.02.005"}],"container-title":["The Journal of Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpath.2782","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/pathsocjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/path.2782","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T12:23:43Z","timestamp":1761049423000},"score":1,"resource":{"primary":{"URL":"https:\/\/pathsocjournals.onlinelibrary.wiley.com\/doi\/10.1002\/path.2782"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,10,11]]},"references-count":18,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2011,1]]}},"alternative-id":["10.1002\/path.2782"],"URL":"https:\/\/doi.org\/10.1002\/path.2782","archive":["Portico"],"relation":{},"ISSN":["0022-3417","1096-9896"],"issn-type":[{"value":"0022-3417","type":"print"},{"value":"1096-9896","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,10,11]]},"assertion":[{"value":"2010-09-01","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2010-09-12","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2010-10-11","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}