{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T06:08:16Z","timestamp":1792649296079,"version":"4.5.0"},"reference-count":50,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2023,3,1]],"date-time":"2023-03-01T00:00:00Z","timestamp":1677628800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,3,1]],"date-time":"2023-03-01T00:00:00Z","timestamp":1677628800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,3,1]],"date-time":"2025-03-01T00:00:00Z","timestamp":1740787200000},"content-version":"vor","delay-in-days":731,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["279131"],"award-info":[{"award-number":["279131"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["CFF SWIATE18G0"],"award-info":[{"award-number":["CFF SWIATE18G0"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01HL144539"],"award-info":[{"award-number":["R01HL144539"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000897","name":"Cystic Fibrosis Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000897","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100011022","name":"Vertex Pharmaceuticals Inc","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100011022","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002426","name":"Fondazione Telethon","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","cysticfibrosisjournal.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Cystic Fibrosis"],"published-print":{"date-parts":[[2023,3]]},"DOI":"10.1016\/j.jcf.2022.12.015","type":"journal-article","created":{"date-parts":[[2023,1,7]],"date-time":"2023-01-07T00:40:41Z","timestamp":1673052041000},"page":"S12-S16","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":11,"special_numbering":"S1","title":["Cell signaling and regulation of CFTR expression in cystic fibrosis cells in the era of high efficiency modulator therapy"],"prefix":"10.1016","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1193-5296","authenticated-orcid":false,"given":"Alessandra","family":"Ghigo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"De Santi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Merrill","family":"Hart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8899-0915","authenticated-orcid":false,"given":"Nilay","family":"Mitash","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agnieszka","family":"Swiatecka-Urban","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jcf.2022.12.015_bib0001","doi-asserted-by":"crossref","DOI":"10.3390\/genes10030235","article-title":"Cell-selective regulation of CFTR gene expression: relevance to gene editing therapeutics","volume":"10","author":"Swahn","year":"2019","journal-title":"Genes (Basel)"},{"key":"10.1016\/j.jcf.2022.12.015_bib0002","doi-asserted-by":"crossref","first-page":"15010","DOI":"10.1038\/nrdp.2015.10","article-title":"Cystic fibrosis","volume":"1","author":"Ratjen","year":"2015","journal-title":"Nat Rev Dis Primers"},{"key":"10.1016\/j.jcf.2022.12.015_bib0003","doi-asserted-by":"crossref","first-page":"2195","DOI":"10.1016\/S0140-6736(20)32542-3","article-title":"Cystic fibrosis","volume":"397","author":"Shteinberg","year":"2021","journal-title":"Lancet"},{"key":"10.1016\/j.jcf.2022.12.015_bib0004","doi-asserted-by":"crossref","DOI":"10.3389\/fphys.2021.690247","article-title":"Role of protein Kinase A-mediated phosphorylation in CFTR channel activity regulation","volume":"12","author":"Della Sala","year":"2021","journal-title":"Front Physiol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0005","doi-asserted-by":"crossref","first-page":"21740","DOI":"10.1073\/pnas.2007910117","article-title":"Simple binding of protein kinase A prior to phosphorylation allows CFTR anion channels to be opened by nucleotides","volume":"117","author":"Mihalyi","year":"2020","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.jcf.2022.12.015_bib0006","doi-asserted-by":"crossref","DOI":"10.3390\/ijms21124524","article-title":"Full rescue of F508del-CFTR processing and function by CFTR modulators can be achieved by removal of two regulatory regions","volume":"21","author":"Uliyakina","year":"2020","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.jcf.2022.12.015_bib0007","doi-asserted-by":"crossref","first-page":"13460","DOI":"10.1038\/s41598-019-49921-4","article-title":"VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level","volume":"9","author":"Cui","year":"2019","journal-title":"Sci Rep"},{"key":"10.1016\/j.jcf.2022.12.015_bib0008","doi-asserted-by":"crossref","first-page":"2599","DOI":"10.1242\/jcs.185629","article-title":"EPAC1 activation by cAMP stabilizes CFTR at the membrane by promoting its interaction with NHERF1","volume":"129","author":"Lobo","year":"2016","journal-title":"J Cell Sci"},{"key":"10.1016\/j.jcf.2022.12.015_bib0009","doi-asserted-by":"crossref","first-page":"19810","DOI":"10.1038\/s41598-021-99184-1","article-title":"Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment","volume":"11","author":"Shaughnessy","year":"2021","journal-title":"Sci Rep"},{"key":"10.1016\/j.jcf.2022.12.015_bib0010","doi-asserted-by":"crossref","first-page":"1128","DOI":"10.1242\/jcs.177907","article-title":"Correctors of mutant CFTR enhance subcortical cAMP-PKA signaling through modulating ezrin phosphorylation and cytoskeleton organization","volume":"129","author":"Abbattiscianni","year":"2016","journal-title":"J Cell Sci"},{"key":"10.1016\/j.jcf.2022.12.015_bib0011","doi-asserted-by":"crossref","first-page":"768","DOI":"10.1183\/13993003.01661-2015","article-title":"beta2-Adrenergic receptor agonists activate CFTR in intestinal organoids and subjects with cystic fibrosis","volume":"48","author":"Vijftigschild","year":"2016","journal-title":"Eur Respir J"},{"key":"10.1016\/j.jcf.2022.12.015_bib0012","doi-asserted-by":"crossref","DOI":"10.1172\/jci.insight.93029","article-title":"Chronic beta2AR stimulation limits CFTR activation in human airway epithelia","volume":"3","author":"Brewington","year":"2018","journal-title":"JCI Insight"},{"key":"10.1016\/j.jcf.2022.12.015_bib0013","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1096\/fj.13-240861","article-title":"Anchored PDE4 regulates chloride conductance in wild-type and DeltaF508-CFTR human airway epithelia","volume":"28","author":"Blanchard","year":"2014","journal-title":"FASEB J"},{"key":"10.1016\/j.jcf.2022.12.015_bib0014","doi-asserted-by":"crossref","DOI":"10.1152\/ajplung.00324.2015","article-title":"The dual phosphodiesterase 3 and 4 inhibitor RPL554 stimulates CFTR and ciliary beating in primary cultures of bronchial epithelia","volume":"310","author":"Turner","year":"2016","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0015","doi-asserted-by":"crossref","first-page":"L908","DOI":"10.1152\/ajplung.00285.2019","article-title":"The dual phosphodiesterase 3\/4 inhibitor RPL554 stimulates rare class III and IV CFTR mutants","volume":"318","author":"Turner","year":"2020","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0016","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.abl6328","article-title":"A PI3Kgamma mimetic peptide triggers CFTR gating, bronchodilation, and reduced inflammation in obstructive airway diseases","volume":"14","author":"Ghigo","year":"2022","journal-title":"Sci Transl Med"},{"key":"10.1016\/j.jcf.2022.12.015_bib0017","doi-asserted-by":"crossref","first-page":"784","DOI":"10.33594\/000000477","article-title":"Phosphodiesterase 8A regulates CFTR activity in airway epithelial cells","volume":"55","author":"Turner","year":"2021","journal-title":"Cell Physiol Biochem"},{"key":"10.1016\/j.jcf.2022.12.015_bib0018","doi-asserted-by":"crossref","first-page":"904","DOI":"10.1038\/s41598-020-79555-w","article-title":"Modulation of cAMP metabolism for CFTR potentiation in human airway epithelial cells","volume":"11","author":"Nguyen","year":"2021","journal-title":"Sci Rep"},{"key":"10.1016\/j.jcf.2022.12.015_bib0019","doi-asserted-by":"crossref","first-page":"12706","DOI":"10.1038\/s41598-019-48971-y","article-title":"Phosphorylation-dependent modulation of CFTR macromolecular signalling complex activity by cigarette smoke condensate in airway epithelia","volume":"9","author":"Schnur","year":"2019","journal-title":"Sci Rep"},{"key":"10.1016\/j.jcf.2022.12.015_bib0020","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.00671-2021","article-title":"The rescue of F508del-CFTR by elexacaftor\/tezacaftor\/ivacaftor (Trikafta) in human airway epithelial cells is underestimated due to the presence of ivacaftor","volume":"59","author":"Becq","year":"2022","journal-title":"Eur Respir J"},{"key":"10.1016\/j.jcf.2022.12.015_bib0021","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1042\/BST20170469","article-title":"Non-coding RNA in cystic fibrosis","volume":"46","author":"Glasgow","year":"2018","journal-title":"Biochem Soc Trans"},{"key":"10.1016\/j.jcf.2022.12.015_bib0022","doi-asserted-by":"crossref","DOI":"10.1126\/sciadv.aao2110","article-title":"How do lncRNAs regulate transcription?","volume":"3","author":"Long","year":"2017","journal-title":"Sci Adv"},{"key":"10.1016\/j.jcf.2022.12.015_bib0023","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1016\/j.biocel.2014.02.022","article-title":"Long noncoding RNA are aberrantly expressed in vivo in the cystic fibrosis bronchial epithelium","volume":"52","author":"McKiernan","year":"2014","journal-title":"Int J Biochem Cell Biol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0024","doi-asserted-by":"crossref","first-page":"218","DOI":"10.3389\/fcimb.2017.00218","article-title":"Bronchial epithelial cells from cystic fibrosis patients express a specific long non-coding RNA signature upon pseudomonas aeruginosa infection","volume":"7","author":"Balloy","year":"2017","journal-title":"Front Cell Infect Microbiol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0025","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1186\/s12931-019-1259-8","article-title":"Comparative analyses of long non-coding RNA profiles in vivo in cystic fibrosis lung airway and parenchyma tissues","volume":"20","author":"Kumar","year":"2019","journal-title":"Respir Res"},{"key":"10.1016\/j.jcf.2022.12.015_bib0026","doi-asserted-by":"crossref","first-page":"1351","DOI":"10.1038\/mt.2016.112","article-title":"Long non-coding RNA BGas regulates the cystic fibrosis transmembrane conductance regulator","volume":"24","author":"Saayman","year":"2016","journal-title":"Mol Ther"},{"key":"10.1016\/j.jcf.2022.12.015_bib0027","doi-asserted-by":"crossref","first-page":"1737","DOI":"10.1016\/j.ymthe.2019.07.002","article-title":"Targeted activation of cystic fibrosis transmembrane conductance regulator","volume":"27","author":"Villamizar","year":"2019","journal-title":"Mol Ther"},{"key":"10.1016\/j.jcf.2022.12.015_bib0028","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1042\/BJ20110672","article-title":"MicroRNA regulation of expression of the cystic fibrosis transmembrane conductance regulator gene","volume":"438","author":"Gillen","year":"2011","journal-title":"Biochem J"},{"key":"10.1016\/j.jcf.2022.12.015_bib0029","doi-asserted-by":"crossref","first-page":"e26601","DOI":"10.1371\/journal.pone.0026601","article-title":"Synergistic post-transcriptional regulation of the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) by miR-101 and miR-494 specific binding","volume":"6","author":"Megiorni","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.jcf.2022.12.015_bib0030","doi-asserted-by":"crossref","first-page":"3354","DOI":"10.4049\/jimmunol.1202960","article-title":"Regulation of cystic fibrosis transmembrane conductance regulator by microRNA-145, -223, and -494 is altered in DeltaF508 cystic fibrosis airway epithelium","volume":"190","author":"Oglesby","year":"2013","journal-title":"J Immunol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0031","doi-asserted-by":"crossref","first-page":"544","DOI":"10.1165\/rcmb.2012-0430OC","article-title":"Post-transcriptional regulation of cystic fibrosis transmembrane conductance regulator expression and function by microRNAs","volume":"49","author":"Ramachandran","year":"2013","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0032","doi-asserted-by":"crossref","first-page":"e50837","DOI":"10.1371\/journal.pone.0050837","article-title":"MiR-101 and miR-144 regulate the expression of the CFTR chloride channel in the lung","volume":"7","author":"Hassan","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.jcf.2022.12.015_bib0033","doi-asserted-by":"crossref","DOI":"10.3390\/ijms20194933","article-title":"Transforming growth factor-beta1 selectively recruits microRNAs to the RNA-induced silencing complex and degrades CFTR mRNA under permissive conditions in human bronchial epithelial cells","volume":"20","author":"Mitash","year":"2019","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.jcf.2022.12.015_bib0034","doi-asserted-by":"crossref","first-page":"1190","DOI":"10.1016\/j.ymthe.2020.02.001","article-title":"Precise targeting of miRNA sites restores CFTR activity in CF bronchial epithelial cells","volume":"28","author":"De Santi","year":"2020","journal-title":"Mol Ther"},{"key":"10.1016\/j.jcf.2022.12.015_bib0035","doi-asserted-by":"crossref","DOI":"10.3390\/molecules22071144","article-title":"Peptide nucleic acids as miRNA target protectors for the treatment of cystic fibrosis","volume":"22","author":"Zarrilli","year":"2017","journal-title":"Molecules"},{"key":"10.1016\/j.jcf.2022.12.015_bib0036","doi-asserted-by":"crossref","DOI":"10.3390\/ijms23169348","article-title":"Combined treatment of bronchial epithelial Calu-3 cells with peptide nucleic acids targeting miR-145-5p and miR-101-3p: synergistic enhancement of the expression of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene","volume":"23","author":"Papi","year":"2022","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.jcf.2022.12.015_bib0037","doi-asserted-by":"crossref","first-page":"179","DOI":"10.2217\/epi-2019-0395","article-title":"Challenges facing microRNA therapeutics for cystic fibrosis lung disease","volume":"12","author":"De Santi","year":"2020","journal-title":"Epigenomics"},{"key":"10.1016\/j.jcf.2022.12.015_bib0038","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1038\/s41392-021-00569-5","article-title":"Targeting circular RNAs as a therapeutic approach: current strategies and challenges","volume":"6","author":"He","year":"2021","journal-title":"Signal Transduct Target Ther"},{"key":"10.1016\/j.jcf.2022.12.015_bib0039","doi-asserted-by":"crossref","first-page":"1443","DOI":"10.1056\/NEJMoa051469","article-title":"Genetic modifiers of lung disease in cystic fibrosis","volume":"353","author":"Drumm","year":"2005","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jcf.2022.12.015_bib0040","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1111\/j.1749-6632.2010.05879.x","article-title":"Modifier genes in Mendelian disorders: the example of cystic fibrosis","volume":"1214","author":"Cutting","year":"2010","journal-title":"Ann N Y Acad Sci"},{"key":"10.1016\/j.jcf.2022.12.015_bib0041","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1016\/j.clim.2006.08.015","article-title":"Polymorphisms of TGF-beta1 in cystic fibrosis patients","volume":"121","author":"Brazova","year":"2006","journal-title":"Clin Immunol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0042","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1002\/ppul.21079","article-title":"Transforming growth factor-beta(1) in bronchoalveolar lavage fluid from children with cystic fibrosis","volume":"44","author":"Harris","year":"2009","journal-title":"Pediatr Pulmonol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0043","doi-asserted-by":"crossref","first-page":"e63167","DOI":"10.1371\/journal.pone.0063167","article-title":"Tgf-beta1 inhibits Cftr biogenesis and prevents functional rescue of DeltaF508-Cftr in primary differentiated human bronchial epithelial cells","volume":"8","author":"Snodgrass","year":"2013","journal-title":"PLoS One"},{"key":"10.1016\/j.jcf.2022.12.015_bib0044","doi-asserted-by":"crossref","first-page":"632","DOI":"10.1164\/rccm.201704-0732OC","article-title":"MicroRNA-145 antagonism reverses TGF-beta inhibition of F508del CFTR correction in airway epithelia","volume":"197","author":"Lutful Kabir","year":"2018","journal-title":"Am J Respir Crit Care Med"},{"key":"10.1016\/j.jcf.2022.12.015_bib0045","article-title":"Tgf-beta downregulation of distinct chloride channels in cystic fibrosis-affected epithelia","volume":"9","author":"Sun","year":"2014","journal-title":"PLoS One"},{"key":"10.1016\/j.jcf.2022.12.015_bib0046","doi-asserted-by":"crossref","first-page":"25710","DOI":"10.1074\/jbc.M115.670885","article-title":"Airway surface dehydration by transforming growth factor beta (TGF-beta) in cystic fibrosis is due to decreased function of a voltage-dependent potassium channel and can be rescued by the drug pirfenidone","volume":"290","author":"Manzanares","year":"2015","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jcf.2022.12.015_bib0047","doi-asserted-by":"crossref","first-page":"L456","DOI":"10.1152\/ajplung.00530.2017","article-title":"Subacute TGFbeta expression drives inflammation, goblet cell hyperplasia, and pulmonary function abnormalities in mice with effects dependent on CFTR function","volume":"315","author":"Kramer","year":"2018","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0048","doi-asserted-by":"crossref","first-page":"15080","DOI":"10.1074\/jbc.M114.563742","article-title":"LMTK2-mediated phosphorylation regulates CFTR endocytosis in human airway epithelial cells","volume":"289","author":"Luz","year":"2014","journal-title":"J Biol Chem"},{"key":"10.1016\/j.jcf.2022.12.015_bib0049","doi-asserted-by":"crossref","DOI":"10.3389\/fonc.2021.596861","article-title":"Differential gene expression analysis reveals global LMTK2 regulatory network and its role in TGF-beta1 signaling","volume":"11","author":"Cruz","year":"2021","journal-title":"Front Oncol"},{"key":"10.1016\/j.jcf.2022.12.015_bib0050","doi-asserted-by":"crossref","first-page":"58","DOI":"10.3389\/fcell.2020.00058","article-title":"TGF-beta1 augments the apical membrane abundance of lemur tyrosine Kinase 2 to inhibit CFTR-mediated chloride transport in human bronchial epithelia","volume":"8","author":"Cruz","year":"2020","journal-title":"Front Cell Dev Biol"}],"container-title":["Journal of Cystic Fibrosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199322014357?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199322014357?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T21:47:42Z","timestamp":1761601662000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1569199322014357"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,3]]},"references-count":50,"alternative-id":["S1569199322014357"],"URL":"https:\/\/doi.org\/10.1016\/j.jcf.2022.12.015","relation":{},"ISSN":["1569-1993"],"issn-type":[{"value":"1569-1993","type":"print"}],"subject":[],"published":{"date-parts":[[2023,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Cell signaling and regulation of CFTR expression in cystic fibrosis cells in the era of high efficiency modulator therapy","name":"articletitle","label":"Article Title"},{"value":"Journal of Cystic Fibrosis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jcf.2022.12.015","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2022 European Cystic Fibrosis Society. Published by Elsevier B.V.","name":"copyright","label":"Copyright"}]}}