{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T14:49:00Z","timestamp":1774363740741,"version":"3.50.1"},"reference-count":43,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2021,8,20]],"date-time":"2021-08-20T00:00:00Z","timestamp":1629417600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001871","name":"Funda\u00e7\u00e3o para a Ci\u00eancia e a Tecnologia","doi-asserted-by":"publisher","award":["iNOVA4Health-UID\/Multi\/04462\/2013"],"award-info":[{"award-number":["iNOVA4Health-UID\/Multi\/04462\/2013"]}],"id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001871","name":"Funda\u00e7\u00e3o para a Ci\u00eancia e a Tecnologia","doi-asserted-by":"publisher","award":["FCT-ANR\/BEX-BID\/0153\/2012"],"award-info":[{"award-number":["FCT-ANR\/BEX-BID\/0153\/2012"]}],"id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001871","name":"Funda\u00e7\u00e3o para a Ci\u00eancia e a Tecnologia","doi-asserted-by":"publisher","award":["PTDC\/BEX-BID\/1411\/2014"],"award-info":[{"award-number":["PTDC\/BEX-BID\/1411\/2014"]}],"id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001871","name":"Funda\u00e7\u00e3o para a Ci\u00eancia e a Tecnologia","doi-asserted-by":"publisher","award":["LISBOA-01-0145-FEDER-022170"],"award-info":[{"award-number":["LISBOA-01-0145-FEDER-022170"]}],"id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Horizon 2020","award":["811087"],"award-info":[{"award-number":["811087"]}]},{"DOI":"10.13039\/501100007044","name":"Sociedade Portuguesa de Nefrologia","doi-asserted-by":"publisher","award":["Projecto de 2015"],"award-info":[{"award-number":["Projecto de 2015"]}],"id":[{"id":"10.13039\/501100007044","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>In autosomal dominant polycystic kidney disease (ADPKD), kidney cyst growth requires the recruitment of CFTR (cystic fibrosis transmembrane conductance regulator), the chloride channel that is defective in cystic fibrosis. We have been studying cyst inflation using the zebrafish Kupffer\u2019s vesicle (KV) as model system because we previously demonstrated that knocking down polycystin 2 (PC2) induced a CFTR-mediated enlargement of the organ. We have now quantified the PC2 knockdown by showing that it causes a 73% reduction in the number of KV cilia expressing PC2. According to the literature, this is an essential event in kidney cystogenesis in ADPKD mice. Additionally, we demonstrated that the PC2 knockdown leads to a significant accumulation of CFTR-GFP at the apical region of the KV cells. Furthermore, we determined that KV enlargement is rescued by the injection of Xenopus pkd2 mRNA and by 100 \u00b5M tolvaptan treatment, the unique and approved pharmacologic approach for ADPKD management. We expected vasopressin V2 receptor antagonist to lower the cAMP levels of KV-lining cells and, thus, to inactivate CFTR. These findings further support the use of the KV as an in vivo model for screening compounds that may prevent cyst enlargement in this ciliopathy, through CFTR inhibition.<\/jats:p>","DOI":"10.3390\/ijms22169013","type":"journal-article","created":{"date-parts":[[2021,8,22]],"date-time":"2021-08-22T21:47:52Z","timestamp":1629668872000},"page":"9013","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Zebrafish Model as a Screen to Prevent Cyst Inflation in Autosomal Dominant Polycystic Kidney Disease"],"prefix":"10.3390","volume":"22","author":[{"given":"In\u00eas","family":"Oliveira","sequence":"first","affiliation":[{"name":"CEDOC, Chronic Diseases Research Center, NOVA Medical School|Faculdade de Ci\u00eancias M\u00e9dicas, Universidade Nova de Lisboa, Campo M\u00e1rtires da P\u00e1tria, 130, 1169-056 Lisboa, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4029-0204","authenticated-orcid":false,"given":"Raquel","family":"Jacinto","sequence":"additional","affiliation":[{"name":"CEDOC, Chronic Diseases Research Center, NOVA Medical School|Faculdade de Ci\u00eancias M\u00e9dicas, Universidade Nova de Lisboa, Campo M\u00e1rtires da P\u00e1tria, 130, 1169-056 Lisboa, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0995-0040","authenticated-orcid":false,"given":"Sara","family":"Pestana","sequence":"additional","affiliation":[{"name":"CEDOC, Chronic Diseases Research Center, NOVA Medical School|Faculdade de Ci\u00eancias M\u00e9dicas, Universidade Nova de Lisboa, Campo M\u00e1rtires da P\u00e1tria, 130, 1169-056 Lisboa, Portugal"}]},{"given":"Fernando","family":"Nolasco","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Centro Hospitalar e Universit\u00e1rio de Lisboa Central, Hospital de Curry Cabral, Rua da Benefic\u00eancia, 8, 1069-166 Lisboa, Portugal"}]},{"given":"Joaquim","family":"Calado","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Centro Hospitalar e Universit\u00e1rio de Lisboa Central, Hospital de Curry Cabral, Rua da Benefic\u00eancia, 8, 1069-166 Lisboa, Portugal"},{"name":"ToxOmics, Center of ToxicoGenomics & Human Health, NOVA Medical School|Faculdade de Ci\u00eancias M\u00e9dicas, Universidade Nova de Lisboa, Campo M\u00e1rtires da P\u00e1tria, 130, 1169-056 Lisboa, Portugal"}]},{"given":"Susana Santos","family":"Lopes","sequence":"additional","affiliation":[{"name":"CEDOC, Chronic Diseases Research Center, NOVA Medical School|Faculdade de Ci\u00eancias M\u00e9dicas, Universidade Nova de Lisboa, Campo M\u00e1rtires da P\u00e1tria, 130, 1169-056 Lisboa, Portugal"}]},{"given":"M\u00f3nica","family":"Roxo-Rosa","sequence":"additional","affiliation":[{"name":"CEDOC, Chronic Diseases Research Center, NOVA Medical School|Faculdade de Ci\u00eancias M\u00e9dicas, Universidade Nova de Lisboa, Campo M\u00e1rtires da P\u00e1tria, 130, 1169-056 Lisboa, Portugal"}]}],"member":"1968","published-online":{"date-parts":[[2021,8,20]]},"reference":[{"key":"ref_1","first-page":"1","article-title":"Bilateral polycystic disease of the kidneys: A follow-up of two hundred and eighty-four patients and their families","volume":"328","author":"Dalgaard","year":"1957","journal-title":"Acta Med. 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