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dc.contributor.author | Gianotti, Ambra | |
dc.contributor.author | Capurro, Valeria | |
dc.contributor.author | Delpiano, Livia | |
dc.contributor.author | Mielczarek, Marcin | |
dc.contributor.author | García Valverde, María | |
dc.contributor.author | Carreira Barral, Israel | |
dc.contributor.author | Ludovico, Alessandra | |
dc.contributor.author | Fiore, Michele | |
dc.contributor.author | Baroni, Debora | |
dc.contributor.author | Moran, Óscar | |
dc.contributor.author | Quesada Pato, Roberto | |
dc.contributor.author | Caci, Emanuela | |
dc.date.accessioned | 2024-01-13T19:36:00Z | |
dc.date.available | 2024-01-13T19:36:00Z | |
dc.date.issued | 2020 | |
dc.identifier.issn | 1422-0067 | |
dc.identifier.uri | http://hdl.handle.net/10259/8329 | |
dc.description.abstract | Cystic fibrosis (CF) is a genetic disease characterized by the lack of cystic fibrosis transmembrane conductance regulator (CFTR) protein expressed in epithelial cells. The resulting defective chloride and bicarbonate secretion and imbalance of the transepithelial homeostasis lead to abnormal airway surface liquid (ASL) composition and properties. The reduced ASL volume impairs ciliary beating with the consequent accumulation of sticky mucus. This situation prevents the normal mucociliary clearance, favouring the survival and proliferation of bacteria and contributing to the genesis of CF lung disease. Here, we have explored the potential of small molecules capable of facilitating the transmembrane transport of chloride and bicarbonate in order to replace the defective transport activity elicited by CFTR in CF airway epithelia. Primary human bronchial epithelial cells obtained from CF and non-CF patients were differentiated into a mucociliated epithelia in order to assess the effects of our compounds on some key properties of ASL. The treatment of these functional models with non-toxic doses of the synthetic anionophores improved the periciliary fluid composition, reducing the fluid re-absorption, correcting the ASL pH and reducing the viscosity of the mucus, thus representing promising drug candidates for CF therapy. | en |
dc.description.sponsorship | This work was financially supported by the European Union’s Horizon 2020 research and innovation programme under grant agreement no. 667079 and from the Italian Cystic Fibrosis Foundation (FFC no. CP/2012 with the contribution of ‘Delegazione FFC di Verona and Imola-Romagna’ and ‘Delegazione FFC di Roma’). | en |
dc.format.mimetype | application/pdf | |
dc.language.iso | eng | es |
dc.publisher | MDPI | es |
dc.relation.ispartof | International Journal of Molecular Sciences. 2020, V. 21, n. 4, 1488 | es |
dc.rights | Atribución 4.0 Internacional | * |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/ | * |
dc.subject | Cystic fibrosis | en |
dc.subject | Anionophore | en |
dc.subject | Bronchial epithelial cells culture | en |
dc.subject | Ion transport | en |
dc.subject | Periciliar mucus properties | en |
dc.subject.other | Medicina | es |
dc.subject.other | Medicine | en |
dc.subject.other | Salud | es |
dc.subject.other | Health | en |
dc.subject.other | Química orgánica | es |
dc.subject.other | Chemistry, Organic | en |
dc.title | Small Molecule Anion Carriers Correct Abnormal Airway Surface Liquid Properties in Cystic Fibrosis Airway Epithelia | en |
dc.type | info:eu-repo/semantics/article | es |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | es |
dc.relation.publisherversion | https://doi.org/10.3390/ijms21041488 | es |
dc.identifier.doi | 10.3390/ijms21041488 | |
dc.identifier.essn | 1422-0067 | |
dc.journal.title | International Journal of Molecular Sciences | es |
dc.volume.number | 21 | es |
dc.issue.number | 4 | es |
dc.page.initial | 1488 | es |
dc.type.hasVersion | info:eu-repo/semantics/publishedVersion | es |