{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,11]],"date-time":"2026-04-11T14:40:27Z","timestamp":1775918427433,"version":"3.50.1"},"reference-count":92,"publisher":"The Company of Biologists","issue":"24","license":[{"start":{"date-parts":[[2023,12,15]],"date-time":"2023-12-15T00:00:00Z","timestamp":1702598400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/www.biologists.com\/user-licence-1-1\/"}],"funder":[{"DOI":"10.13039\/100000057","name":"National Institute of General Medical Sciences","doi-asserted-by":"crossref","award":["R01GM140115"],"award-info":[{"award-number":["R01GM140115"]}],"id":[{"id":"10.13039\/100000057","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"crossref","award":["R01-DK108005"],"award-info":[{"award-number":["R01-DK108005"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,12,15]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Mutations that disrupt centrosome biogenesis or function cause congenital kidney developmental defects and fibrocystic pathologies. Yet how centrosome dysfunction results in the kidney disease phenotypes remains unknown. Here, we examined the consequences of conditional knockout of the ciliopathy gene Cep120, essential for centrosome duplication, in the nephron and collecting duct progenitor niches of the mouse embryonic kidney. Cep120 loss led to reduced abundance of both cap mesenchyme and ureteric bud populations, due to a combination of delayed mitosis, increased apoptosis and premature differentiation of progenitor cells. These defects resulted in dysplastic kidneys at birth, which rapidly formed cysts, displayed increased interstitial fibrosis and decline in kidney function. RNA sequencing of embryonic and postnatal kidneys from Cep120-null mice identified changes in the pathways essential for development, fibrosis and cystogenesis. Our study defines the cellular and developmental defects caused by centrosome dysfunction during kidney morphogenesis and identifies new therapeutic targets for patients with renal centrosomopathies.<\/jats:p>","DOI":"10.1242\/dev.201976","type":"journal-article","created":{"date-parts":[[2023,11,20]],"date-time":"2023-11-20T12:23:25Z","timestamp":1700483005000},"source":"Crossref","is-referenced-by-count":6,"title":["Aberrant centrosome biogenesis disrupts nephron and collecting duct progenitor growth and fate resulting in fibrocystic kidney disease"],"prefix":"10.1242","volume":"150","author":[{"given":"Tao","family":"Cheng","sequence":"first","affiliation":[{"name":"Washington University in St Louis 1 Department of Medicine, Division of Nephrology , , St. Louis, MO 63110 , USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chidera","family":"Agwu","sequence":"additional","affiliation":[{"name":"Washington University in St Louis 1 Department of Medicine, Division of Nephrology , , St. Louis, MO 63110 , USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kyuhwan","family":"Shim","sequence":"additional","affiliation":[{"name":"Washington University in St Louis 1 Department of Medicine, Division of Nephrology , , St. Louis, MO 63110 , USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Baolin","family":"Wang","sequence":"additional","affiliation":[{"name":"Weill Medical College of Cornell University 2 Department of Genetic Medicine , , New York, NY 10065 , USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2804-127X","authenticated-orcid":false,"given":"Sanjay","family":"Jain","sequence":"additional","affiliation":[{"name":"Washington University in St Louis 1 Department of Medicine, Division of Nephrology , , St. Louis, MO 63110 , USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8129-7464","authenticated-orcid":false,"given":"Moe R.","family":"Mahjoub","sequence":"additional","affiliation":[{"name":"Washington University in St Louis 1 Department of Medicine, Division of Nephrology , , St. Louis, MO 63110 , USA"},{"name":"Washington University in St Louis 3 Department of Cell Biology and Physiology , , St. Louis, MO 63110 , USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"237","published-online":{"date-parts":[[2023,12,21]]},"reference":[{"key":"2023122116532848800_DEV201976C1","doi-asserted-by":"publisher","first-page":"922428","DOI":"10.3389\/fmolb.2022.922428","article-title":"Molecular mechanisms of epigenetic regulation, inflammation, and cell death in ADPKD","volume":"9","author":"Agborbesong","year":"2022","journal-title":"Front. 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