Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis
Fibrosi quística; Marcadors predictius; Models d'aparell respiratori
| Autores: | , , , , , |
|---|---|
| Tipo de recurso: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2021 |
| País: | España |
| Institución: | Departament de Salut de la Generalitat de Catalunya (DS) |
| Repositorio: | Scientia. Dipòsit d'Informació Digital del Departament de Salut |
| OAI Identifier: | oai:scientiasalut.gencat.cat:11351/7252 |
| Acceso en línea: | https://hdl.handle.net/11351/7252 |
| Access Level: | acceso abierto |
| Palabra clave: | Fibrosi quística - Fisiologia patològica Cultiu cel·lular - Investigació Fibrosi quística - Tractament ANATOMY::Cells::Cells, Cultured::Spheroids, Cellular Other subheadings::Other subheadings::Other subheadings::/metabolism DISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic Fibrosis Other subheadings::Other subheadings::Other subheadings::/pathology ANATOMÍA::células::células cultivadas::esferoides celulares Otros calificadores::Otros calificadores::Otros calificadores::/metabolismo ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quística Otros calificadores::Otros calificadores::Otros calificadores::/patología |
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Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosisCalucho Prim, MaiteGartner Tizzano, SilviaBarranco Bartolomé, PaulaFernández Álvarez, PaulaGarcía Pérez, RaquelTizzano Ferrari, Eduardo FidelFibrosi quística - Fisiologia patològicaCultiu cel·lular - InvestigacióFibrosi quística - TractamentANATOMY::Cells::Cells, Cultured::Spheroids, CellularOther subheadings::Other subheadings::Other subheadings::/metabolismDISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic FibrosisOther subheadings::Other subheadings::Other subheadings::/pathologyANATOMÍA::células::células cultivadas::esferoides celularesOtros calificadores::Otros calificadores::Otros calificadores::/metabolismoENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quísticaOtros calificadores::Otros calificadores::Otros calificadores::/patologíaFibrosi quística; Marcadors predictius; Models d'aparell respiratoriFibrosis quística; Marcadores predictivos; Modelos del sistema respiratorioCystic fibrosis; Predictive markers; Respiratory system modelsThe availability of a simple, robust and non-invasive in vitro airway model would be useful to study the functionality of the cystic fibrosis transmembrane regulator (CFTR) protein and to personalize modulator therapy for cystic fibrosis (CF) patients. Our aim was to validate a CFTR functional study using nasospheroids, a patient-derived nasal cell 3D-culture. We performed live-cell experiments in nasospheroids obtained from wild-type individuals and CF patients with different genotypes and phenotypes. We extended the existing method and expanded the analysis to upgrade measurements of CFTR activity using forskolin-induced shrinking. We also tested modulator drugs in CF samples. Immobilizing suspended-nasospheroids provided a high number of samples for live-cell imaging. The diversity observed in basal sizes of nasospheroids did not affect the functional analysis of CFTR. Statistical analysis with our method was simple, making this protocol easy to reproduce. Moreover, we implemented the measurement of inner fluid reservoir areas to further differentiate CFTR functionality. In summary, this rapid methodology is helpful to analyse response to modulators in CF samples to allow individualized treatment for CF patients.This work was supported by a grant from Fundación Mutua Madrileña (Rare disorders, MM/XIV/RECERCA/2017/TIZZANO) to E.F.T. and Fundació Arcadi to S.G. and supporting M.C. and P.B.; M.C. was partially supported by Fundació Daniel Bravo Andreu.Nature ResearchInstitut Català de la Salut[Calucho M, Barranco P, Fernández-Álvarez P, Tizzano EF] Grup de Recerca en Medicina Genètica, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Servei de Genètica Clínica i Molecular, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Gartner S] Unitat de Fibrosi Quística, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Pérez RG] Universitat Autònoma de Barcelona, Bellaterra, SpainVall d'Hebron Barcelona Hospital Campus202220222021info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfapplication/pdfhttps://hdl.handle.net/11351/7252Scientiareponame:Scientia. Dipòsit d'Informació Digital del Departament de Salutinstname:Departament de Salut de la Generalitat de Catalunya (DS)InglésScientific Reports;11https://doi.org/10.1038/s41598-021-94798-xAttribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:scientiasalut.gencat.cat:11351/72522026-06-12T09:38:37Z |
| dc.title.none.fl_str_mv |
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis |
| title |
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis |
| spellingShingle |
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis Calucho Prim, Maite Fibrosi quística - Fisiologia patològica Cultiu cel·lular - Investigació Fibrosi quística - Tractament ANATOMY::Cells::Cells, Cultured::Spheroids, Cellular Other subheadings::Other subheadings::Other subheadings::/metabolism DISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic Fibrosis Other subheadings::Other subheadings::Other subheadings::/pathology ANATOMÍA::células::células cultivadas::esferoides celulares Otros calificadores::Otros calificadores::Otros calificadores::/metabolismo ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quística Otros calificadores::Otros calificadores::Otros calificadores::/patología |
| title_short |
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis |
| title_full |
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis |
| title_fullStr |
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis |
| title_full_unstemmed |
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis |
| title_sort |
Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis |
| dc.creator.none.fl_str_mv |
Calucho Prim, Maite Gartner Tizzano, Silvia Barranco Bartolomé, Paula Fernández Álvarez, Paula García Pérez, Raquel Tizzano Ferrari, Eduardo Fidel |
| author |
Calucho Prim, Maite |
| author_facet |
Calucho Prim, Maite Gartner Tizzano, Silvia Barranco Bartolomé, Paula Fernández Álvarez, Paula García Pérez, Raquel Tizzano Ferrari, Eduardo Fidel |
| author_role |
author |
| author2 |
Gartner Tizzano, Silvia Barranco Bartolomé, Paula Fernández Álvarez, Paula García Pérez, Raquel Tizzano Ferrari, Eduardo Fidel |
| author2_role |
author author author author author |
| dc.contributor.none.fl_str_mv |
Institut Català de la Salut [Calucho M, Barranco P, Fernández-Álvarez P, Tizzano EF] Grup de Recerca en Medicina Genètica, Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Servei de Genètica Clínica i Molecular, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Gartner S] Unitat de Fibrosi Quística, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Pérez RG] Universitat Autònoma de Barcelona, Bellaterra, Spain Vall d'Hebron Barcelona Hospital Campus |
| dc.subject.none.fl_str_mv |
Fibrosi quística - Fisiologia patològica Cultiu cel·lular - Investigació Fibrosi quística - Tractament ANATOMY::Cells::Cells, Cultured::Spheroids, Cellular Other subheadings::Other subheadings::Other subheadings::/metabolism DISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic Fibrosis Other subheadings::Other subheadings::Other subheadings::/pathology ANATOMÍA::células::células cultivadas::esferoides celulares Otros calificadores::Otros calificadores::Otros calificadores::/metabolismo ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quística Otros calificadores::Otros calificadores::Otros calificadores::/patología |
| topic |
Fibrosi quística - Fisiologia patològica Cultiu cel·lular - Investigació Fibrosi quística - Tractament ANATOMY::Cells::Cells, Cultured::Spheroids, Cellular Other subheadings::Other subheadings::Other subheadings::/metabolism DISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic Fibrosis Other subheadings::Other subheadings::Other subheadings::/pathology ANATOMÍA::células::células cultivadas::esferoides celulares Otros calificadores::Otros calificadores::Otros calificadores::/metabolismo ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quística Otros calificadores::Otros calificadores::Otros calificadores::/patología |
| description |
Fibrosi quística; Marcadors predictius; Models d'aparell respiratori |
| publishDate |
2021 |
| dc.date.none.fl_str_mv |
2021 2022 2022 |
| dc.type.none.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
| format |
article |
| status_str |
publishedVersion |
| dc.identifier.none.fl_str_mv |
https://hdl.handle.net/11351/7252 |
| url |
https://hdl.handle.net/11351/7252 |
| dc.language.none.fl_str_mv |
Inglés |
| language_invalid_str_mv |
Inglés |
| dc.relation.none.fl_str_mv |
Scientific Reports;11 https://doi.org/10.1038/s41598-021-94798-x |
| dc.rights.none.fl_str_mv |
Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess |
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Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ |
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openAccess |
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application/pdf application/pdf |
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Nature Research |
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Nature Research |
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Scientia reponame:Scientia. Dipòsit d'Informació Digital del Departament de Salut instname:Departament de Salut de la Generalitat de Catalunya (DS) |
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Departament de Salut de la Generalitat de Catalunya (DS) |
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Scientia. Dipòsit d'Informació Digital del Departament de Salut |
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Scientia. Dipòsit d'Informació Digital del Departament de Salut |
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15,811543 |