{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,5]],"date-time":"2026-04-05T10:12:36Z","timestamp":1775383956003,"version":"3.50.1"},"reference-count":38,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,2,15]],"date-time":"2022-02-15T00:00:00Z","timestamp":1644883200000},"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":[[2022,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>WD repeat domain 19 (Wdr19) is a major component of the intraflagellar transport (IFT) machinery, which is involved in the function of primary cilia. However, the effects of <jats:italic>Wdr19<\/jats:italic> on primary cilia formation, cystogenesis, and polycystic kidney disease (PKD) progression remain unclear. To study these effects, we generated three lines of kidney\u2010specific conditional knockout mice: <jats:italic>Wdr19<\/jats:italic>\u2010knockout (<jats:italic>Wdr19<\/jats:italic>\u2010KO, <jats:italic>Wdr19<\/jats:italic><jats:sup><jats:italic>f\/\u2212<\/jats:italic><\/jats:sup>::<jats:italic>Cdh16\u2010Cre<\/jats:italic><jats:sup><jats:italic>Tg\/0<\/jats:italic><\/jats:sup>), <jats:italic>Pkd1<\/jats:italic>\u2010knockout (<jats:italic>Pkd1<\/jats:italic>\u2010KO, <jats:italic>Pkd1<\/jats:italic><jats:sup><jats:italic>f\/\u2212<\/jats:italic><\/jats:sup>::<jats:italic>Cdh16\u2010Cre<\/jats:italic><jats:sup><jats:italic>Tg\/0<\/jats:italic><\/jats:sup>), and <jats:italic>Wdr19<\/jats:italic>\/<jats:italic>Pkd1\u2010<\/jats:italic>double knockout (<jats:italic>Wdr19<\/jats:italic>&amp;<jats:italic>Pkd1<\/jats:italic>\u2010dKO, <jats:italic>Wdr19<\/jats:italic><jats:sup><jats:italic>f\/\u2212<\/jats:italic><\/jats:sup><jats:italic>;Pkd1<\/jats:italic><jats:sup><jats:italic>f\/\u2212<\/jats:italic><\/jats:sup>::<jats:italic>Cdh16\u2010Cre<\/jats:italic><jats:sup><jats:italic>Tg\/0<\/jats:italic><\/jats:sup>) mice. Ultrastructural analysis using transmission electron microscopy (TEM) indicated that the primary cilia were almost absent at postnatal day 10 in <jats:italic>Wdr19<\/jats:italic>\u2010KO mice compared with <jats:italic>Pkd1<\/jats:italic>\u2010KO and wild\u2010type (WT) mice. However, the primary cilia appeared structurally normal even if malfunctional in <jats:italic>Pkd1<\/jats:italic>\u2010deficient cysts. The <jats:italic>Pkd1<\/jats:italic>\u2010KO mice had the most severe PKD progression, including the shortest lifespan (14\u2009days) and the largest renal cysts, among the three knockout lines. Thus, the molecular mechanism of renal cystogenesis in <jats:italic>Wdr19<\/jats:italic>\u2010KO mice (primary cilia abrogation) was different from that in <jats:italic>Pkd1<\/jats:italic>\u2010KO mice (primary cilia malfunction). In summary, <jats:italic>Wdr19<\/jats:italic> deficiency leads to primary cilia abrogation and renal cyst formation. Wdr19 is primarily proposed to participate in retrograde IFT and to be crucial for the construction of primary cilia, which are critical organelles for tubulogenesis in the developing kidneys. \u00a9 2022 The Pathological Society of Great Britain and Ireland.<\/jats:p>","DOI":"10.1002\/path.5863","type":"journal-article","created":{"date-parts":[[2022,1,10]],"date-time":"2022-01-10T12:03:08Z","timestamp":1641816188000},"page":"5-16","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Large deletion of <i>Wdr19<\/i> in developing renal tubules disrupts primary ciliogenesis, leading to polycystic kidney disease in mice"],"prefix":"10.1002","volume":"257","author":[{"given":"Shang\u2010Shiuan","family":"Yu","sequence":"first","affiliation":[{"name":"Institute of Clinical Medicine, College of Medicine National Cheng Kung University  Tainan Taiwan"},{"name":"National Laboratory Animal Center National Applied Research 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