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dc.contributorVall d'Hebron Barcelona Hospital Campus
dc.contributor.authorCalucho Prim, Maite
dc.contributor.authorGartner Tizzano, Silvia
dc.contributor.authorBarranco Bartolomé, Paula
dc.contributor.authorFernández Álvarez, Paula
dc.contributor.authorGarcía Pérez, Raquel
dc.contributor.authorTizzano Ferrari, Eduardo Fidel
dc.date.accessioned2022-03-23T12:01:15Z
dc.date.available2022-03-23T12:01:15Z
dc.date.issued2021-07-30
dc.identifier.citationCalucho M, Gartner S, Barranco P, Fernández-Álvarez P, Pérez RG, Tizzano EF. Validation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis. Sci Rep. 2021 Jul 30;11:15511.
dc.identifier.issn2045-2322
dc.identifier.urihttp://hdl.handle.net/11351/7252
dc.descriptionCystic fibrosis; Predictive markers; Respiratory system models
dc.description.abstractThe 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.
dc.language.isoeng
dc.publisherNature Research
dc.relation.ispartofseriesScientific Reports;11
dc.rightsAttribution 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.sourceScientia
dc.subjectFibrosi quística - Fisiologia patològica
dc.subjectCultiu cel·lular - Investigació
dc.subjectFibrosi quística - Tractament
dc.subject.meshSpheroids, Cellular
dc.subject.mesh/metabolism
dc.subject.meshCystic Fibrosis
dc.subject.mesh/pathology
dc.titleValidation of nasospheroids to assay CFTR functionality and modulator responses in cystic fibrosis
dc.typeinfo:eu-repo/semantics/article
dc.identifier.doi10.1038/s41598-021-94798-x
dc.subject.decsesferoides celulares
dc.subject.decs/metabolismo
dc.subject.decsfibrosis quística
dc.subject.decs/patología
dc.relation.publishversionhttps://doi.org/10.1038/s41598-021-94798-x
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.audienceProfessionals
dc.contributor.organismesInstitut Català de la Salut
dc.contributor.authoraffiliation[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
dc.identifier.pmid34330959
dc.identifier.wos000683318500005
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


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