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Differential Effects of Oleuropein and Hydroxytyrosol on Aggregation and Stability of CFTR NBD1-ΔF508 Domain
Review

Three-Dimensional Airway Spheroids and Organoids for Cystic Fibrosis Research

Department of Medical and Surgical Science, University of Foggia, 71122 Foggia, Italy
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Author to whom correspondence should be addressed.
Academic Editor: Cesar A. Moran
J. Respir. 2021, 1(4), 229-247; https://doi.org/10.3390/jor1040022
Received: 31 August 2021 / Revised: 16 September 2021 / Accepted: 28 September 2021 / Published: 7 October 2021
Cystic fibrosis (CF) is an autosomal recessive multi-organ disease caused by mutations in the CF Transmembrane Conductance Regulator (CFTR) gene, with morbidity and mortality primacy related to the lung disease. The CFTR protein, a chloride/bicarbonate channel, is expressed at the apical side of airway epithelial cells and is mainly involved in appropriate ion and fluid transport across the epithelium. Although many animal and cellular models have been developed to study the pathophysiological consequences of the lack/dysfunction of CFTR, only the three-dimensional (3D) structures termed “spheroids” and “organoids” can enable the reconstruction of airway mucosa to model organ development, disease pathophysiology, and drug screening. Airway spheroids and organoids can be derived from different sources, including adult lungs and induced pluripotent stem cells (iPSCs), each with its advantages and limits. Here, we review the major features of airway spheroids and organoids, anticipating that their potential in the CF field has not been fully shown. Further work is mandatory to understand whether they can accomplish better outcomes than other culture conditions of airway epithelial cells for CF personalized therapies and tissue engineering aims. View Full-Text
Keywords: cystic fibrosis; cystic fibrosis transmembrane conductance regulator; drug screening; human nasal cells; human bronchial cells; induced pluripotent stem cells; model disease; CFTR modulators cystic fibrosis; cystic fibrosis transmembrane conductance regulator; drug screening; human nasal cells; human bronchial cells; induced pluripotent stem cells; model disease; CFTR modulators
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MDPI and ACS Style

Laselva, O.; Conese, M. Three-Dimensional Airway Spheroids and Organoids for Cystic Fibrosis Research. J. Respir. 2021, 1, 229-247. https://doi.org/10.3390/jor1040022

AMA Style

Laselva O, Conese M. Three-Dimensional Airway Spheroids and Organoids for Cystic Fibrosis Research. Journal of Respiration. 2021; 1(4):229-247. https://doi.org/10.3390/jor1040022

Chicago/Turabian Style

Laselva, Onofrio, and Massimo Conese. 2021. "Three-Dimensional Airway Spheroids and Organoids for Cystic Fibrosis Research" Journal of Respiration 1, no. 4: 229-247. https://doi.org/10.3390/jor1040022

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