Zebrafish model as a screen to prevent cyst inflation in autosomal dominant polycystic kidney disease
Autor(a) principal: | |
---|---|
Data de Publicação: | 2021 |
Outros Autores: | , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10362/125091 |
Resumo: | Funding Information: Funding: This work was supported by Sociedade Portuguesa de Nefrologia, iNOVA4Health-UID/Multi/04462/2013 (a program financially supported by Fundação para a Ciência e Tecnolo-gia (FCT)/Ministério da Educação e Ciência, through national funds and co-funded by FEDER under the PT2020 Partnership Agreement) and FCT-ANR/BEX-BID/0153/2012 and PTDC/BEX-BID/1411/2014 research grants. M.R.R. was supported by national funds through FCT, first with an iNOVA post-doctoral fellowship and, more recently, with a contract in the context of the celebration of the program contract foreseen in the numbers 4, 5, and 6 of article 23.◦ of D.L. no. 57/2016 of 29 August, as amended by Law no. 57/2017 of 19 July. S.S.L. had a FCT-Investigator contract, followed by NOVA NMS contracts and, at the moment, a FCT CEEC (Concurso Estímulo ao Emprego Científico) contract, as principal investigator. This article is supported by the LYSOCIL project. This project has received funding from the European Union’s Horizon 2020 research and innovation programme under grant agreement No 811087. Zebrafish used as an animal model were reproduced and maintained in the CEDOC Fish Facility, with the support from the research infrastructure Congento, co-financed by Lisboa Regional Operational Programme (Lisboa2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (ERDF) and FCT under the project LISBOA-01-0145-FEDER-022170. Funding Information: This work was supported by Sociedade Portuguesa de Nefrologia, iNOVA4Health-UID/Multi/04462/2013 (a program financially supported by Funda??o para a Ci?ncia e Tecnolo-gia (FCT)/Minist?rio da Educa??o e Ci?ncia, through national funds and co-funded by FEDER under the PT2020 Partnership Agreement) and FCT-ANR/BEX-BID/0153/2012 and PTDC/BEX-BID/1411/2014 research grants. M.R.R. was supported by national funds through FCT, first with an iNOVA post-doctoral fellowship and, more recently, with a contract in the context of the celebration of the program contract foreseen in the numbers 4, 5, and 6 of article 23.? of D.L. no. 57/2016 of 29 August, as amended by Law no. 57/2017 of 19 July. S.S.L. had a FCT-Investigator contract, followed by NOVA NMS contracts and, at the moment, a FCT CEEC (Concurso Est?mulo ao Emprego Cient?fico) contract, as principal investigator. This article is supported by the LYSOCIL project. This project has received funding from the European Union?s Horizon 2020 research and innovation programme under grant agreement No 811087. Zebrafish used as an animal model were reproduced and maintained in the CEDOC Fish Facility, with the support from the research infrastructure Congento, co-financed by Lisboa Regional Operational Programme (Lisboa2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (ERDF) and FCT under the project LISBOA-01-0145-FEDER-022170. Publisher Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. |
id |
RCAP_7d2cd3ac20abf288088b906f65cb5d96 |
---|---|
oai_identifier_str |
oai:run.unl.pt:10362/125091 |
network_acronym_str |
RCAP |
network_name_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository_id_str |
7160 |
spelling |
Zebrafish model as a screen to prevent cyst inflation in autosomal dominant polycystic kidney diseaseAutosomal dominant polycystic kidney disease (ADPKD)Cystic fibrosis transmembrane conductance regulator (CFTR)Kupffer’s vesicle (KV)Polycystin-2 (PC2)CatalysisMolecular BiologySpectroscopyComputer Science ApplicationsPhysical and Theoretical ChemistryOrganic ChemistryInorganic ChemistryFunding Information: Funding: This work was supported by Sociedade Portuguesa de Nefrologia, iNOVA4Health-UID/Multi/04462/2013 (a program financially supported by Fundação para a Ciência e Tecnolo-gia (FCT)/Ministério da Educação e Ciência, through national funds and co-funded by FEDER under the PT2020 Partnership Agreement) and FCT-ANR/BEX-BID/0153/2012 and PTDC/BEX-BID/1411/2014 research grants. M.R.R. was supported by national funds through FCT, first with an iNOVA post-doctoral fellowship and, more recently, with a contract in the context of the celebration of the program contract foreseen in the numbers 4, 5, and 6 of article 23.◦ of D.L. no. 57/2016 of 29 August, as amended by Law no. 57/2017 of 19 July. S.S.L. had a FCT-Investigator contract, followed by NOVA NMS contracts and, at the moment, a FCT CEEC (Concurso Estímulo ao Emprego Científico) contract, as principal investigator. This article is supported by the LYSOCIL project. This project has received funding from the European Union’s Horizon 2020 research and innovation programme under grant agreement No 811087. Zebrafish used as an animal model were reproduced and maintained in the CEDOC Fish Facility, with the support from the research infrastructure Congento, co-financed by Lisboa Regional Operational Programme (Lisboa2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (ERDF) and FCT under the project LISBOA-01-0145-FEDER-022170. Funding Information: This work was supported by Sociedade Portuguesa de Nefrologia, iNOVA4Health-UID/Multi/04462/2013 (a program financially supported by Funda??o para a Ci?ncia e Tecnolo-gia (FCT)/Minist?rio da Educa??o e Ci?ncia, through national funds and co-funded by FEDER under the PT2020 Partnership Agreement) and FCT-ANR/BEX-BID/0153/2012 and PTDC/BEX-BID/1411/2014 research grants. M.R.R. was supported by national funds through FCT, first with an iNOVA post-doctoral fellowship and, more recently, with a contract in the context of the celebration of the program contract foreseen in the numbers 4, 5, and 6 of article 23.? of D.L. no. 57/2016 of 29 August, as amended by Law no. 57/2017 of 19 July. S.S.L. had a FCT-Investigator contract, followed by NOVA NMS contracts and, at the moment, a FCT CEEC (Concurso Est?mulo ao Emprego Cient?fico) contract, as principal investigator. This article is supported by the LYSOCIL project. This project has received funding from the European Union?s Horizon 2020 research and innovation programme under grant agreement No 811087. Zebrafish used as an animal model were reproduced and maintained in the CEDOC Fish Facility, with the support from the research infrastructure Congento, co-financed by Lisboa Regional Operational Programme (Lisboa2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (ERDF) and FCT under the project LISBOA-01-0145-FEDER-022170. Publisher Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland.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’s 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 knock-down 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 µM 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.Centro de Estudos de Doenças Crónicas (CEDOC)NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)Centre for Toxicogenomics and Human Health (ToxOmics)RUNOliveira, InêsJacinto, RaquelPestana, SaraNolasco, FernandoCalado, JoaquimLopes, Susana SantosRoxo-Rosa, Mónica2021-09-24T02:24:55Z2021-08-022021-08-02T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10362/125091eng1661-6596PURE: 33458999https://doi.org/10.3390/ijms22169013info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2024-03-11T05:06:17Zoai:run.unl.pt:10362/125091Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T03:45:39.512257Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Zebrafish model as a screen to prevent cyst inflation in autosomal dominant polycystic kidney disease |
title |
Zebrafish model as a screen to prevent cyst inflation in autosomal dominant polycystic kidney disease |
spellingShingle |
Zebrafish model as a screen to prevent cyst inflation in autosomal dominant polycystic kidney disease Oliveira, Inês Autosomal dominant polycystic kidney disease (ADPKD) Cystic fibrosis transmembrane conductance regulator (CFTR) Kupffer’s vesicle (KV) Polycystin-2 (PC2) Catalysis Molecular Biology Spectroscopy Computer Science Applications Physical and Theoretical Chemistry Organic Chemistry Inorganic Chemistry |
title_short |
Zebrafish model as a screen to prevent cyst inflation in autosomal dominant polycystic kidney disease |
title_full |
Zebrafish model as a screen to prevent cyst inflation in autosomal dominant polycystic kidney disease |
title_fullStr |
Zebrafish model as a screen to prevent cyst inflation in autosomal dominant polycystic kidney disease |
title_full_unstemmed |
Zebrafish model as a screen to prevent cyst inflation in autosomal dominant polycystic kidney disease |
title_sort |
Zebrafish model as a screen to prevent cyst inflation in autosomal dominant polycystic kidney disease |
author |
Oliveira, Inês |
author_facet |
Oliveira, Inês Jacinto, Raquel Pestana, Sara Nolasco, Fernando Calado, Joaquim Lopes, Susana Santos Roxo-Rosa, Mónica |
author_role |
author |
author2 |
Jacinto, Raquel Pestana, Sara Nolasco, Fernando Calado, Joaquim Lopes, Susana Santos Roxo-Rosa, Mónica |
author2_role |
author author author author author author |
dc.contributor.none.fl_str_mv |
Centro de Estudos de Doenças Crónicas (CEDOC) NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM) Centre for Toxicogenomics and Human Health (ToxOmics) RUN |
dc.contributor.author.fl_str_mv |
Oliveira, Inês Jacinto, Raquel Pestana, Sara Nolasco, Fernando Calado, Joaquim Lopes, Susana Santos Roxo-Rosa, Mónica |
dc.subject.por.fl_str_mv |
Autosomal dominant polycystic kidney disease (ADPKD) Cystic fibrosis transmembrane conductance regulator (CFTR) Kupffer’s vesicle (KV) Polycystin-2 (PC2) Catalysis Molecular Biology Spectroscopy Computer Science Applications Physical and Theoretical Chemistry Organic Chemistry Inorganic Chemistry |
topic |
Autosomal dominant polycystic kidney disease (ADPKD) Cystic fibrosis transmembrane conductance regulator (CFTR) Kupffer’s vesicle (KV) Polycystin-2 (PC2) Catalysis Molecular Biology Spectroscopy Computer Science Applications Physical and Theoretical Chemistry Organic Chemistry Inorganic Chemistry |
description |
Funding Information: Funding: This work was supported by Sociedade Portuguesa de Nefrologia, iNOVA4Health-UID/Multi/04462/2013 (a program financially supported by Fundação para a Ciência e Tecnolo-gia (FCT)/Ministério da Educação e Ciência, through national funds and co-funded by FEDER under the PT2020 Partnership Agreement) and FCT-ANR/BEX-BID/0153/2012 and PTDC/BEX-BID/1411/2014 research grants. M.R.R. was supported by national funds through FCT, first with an iNOVA post-doctoral fellowship and, more recently, with a contract in the context of the celebration of the program contract foreseen in the numbers 4, 5, and 6 of article 23.◦ of D.L. no. 57/2016 of 29 August, as amended by Law no. 57/2017 of 19 July. S.S.L. had a FCT-Investigator contract, followed by NOVA NMS contracts and, at the moment, a FCT CEEC (Concurso Estímulo ao Emprego Científico) contract, as principal investigator. This article is supported by the LYSOCIL project. This project has received funding from the European Union’s Horizon 2020 research and innovation programme under grant agreement No 811087. Zebrafish used as an animal model were reproduced and maintained in the CEDOC Fish Facility, with the support from the research infrastructure Congento, co-financed by Lisboa Regional Operational Programme (Lisboa2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (ERDF) and FCT under the project LISBOA-01-0145-FEDER-022170. Funding Information: This work was supported by Sociedade Portuguesa de Nefrologia, iNOVA4Health-UID/Multi/04462/2013 (a program financially supported by Funda??o para a Ci?ncia e Tecnolo-gia (FCT)/Minist?rio da Educa??o e Ci?ncia, through national funds and co-funded by FEDER under the PT2020 Partnership Agreement) and FCT-ANR/BEX-BID/0153/2012 and PTDC/BEX-BID/1411/2014 research grants. M.R.R. was supported by national funds through FCT, first with an iNOVA post-doctoral fellowship and, more recently, with a contract in the context of the celebration of the program contract foreseen in the numbers 4, 5, and 6 of article 23.? of D.L. no. 57/2016 of 29 August, as amended by Law no. 57/2017 of 19 July. S.S.L. had a FCT-Investigator contract, followed by NOVA NMS contracts and, at the moment, a FCT CEEC (Concurso Est?mulo ao Emprego Cient?fico) contract, as principal investigator. This article is supported by the LYSOCIL project. This project has received funding from the European Union?s Horizon 2020 research and innovation programme under grant agreement No 811087. Zebrafish used as an animal model were reproduced and maintained in the CEDOC Fish Facility, with the support from the research infrastructure Congento, co-financed by Lisboa Regional Operational Programme (Lisboa2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (ERDF) and FCT under the project LISBOA-01-0145-FEDER-022170. Publisher Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. |
publishDate |
2021 |
dc.date.none.fl_str_mv |
2021-09-24T02:24:55Z 2021-08-02 2021-08-02T00:00:00Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/10362/125091 |
url |
http://hdl.handle.net/10362/125091 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
1661-6596 PURE: 33458999 https://doi.org/10.3390/ijms22169013 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.source.none.fl_str_mv |
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
instname_str |
Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
instacron_str |
RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
collection |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
repository.mail.fl_str_mv |
|
_version_ |
1799138061207994368 |