{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T05:26:53Z","timestamp":1793424413860,"version":"4.5.0"},"reference-count":14,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/501100006282","name":"King Baudouin Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006282","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004040","name":"KU Leuven","doi-asserted-by":"publisher","award":["C24M\/21\/041"],"award-info":[{"award-number":["C24M\/21\/041"]}],"id":[{"id":"10.13039\/501100004040","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":[[2026,3]]},"DOI":"10.1016\/j.jcf.2025.12.018","type":"journal-article","created":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T20:25:35Z","timestamp":1767212735000},"page":"314-318","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":3,"title":["Rethinking CFTR variant responsiveness: Differential responses to vanzacaftor and elexacaftor"],"prefix":"10.1016","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7076-0767","authenticated-orcid":false,"given":"Noelia","family":"Rodriguez Mier","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3124-887X","authenticated-orcid":false,"given":"Isabelle","family":"Callebaut","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5576-9517","authenticated-orcid":false,"given":"Marijke","family":"Proesmans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7242-0747","authenticated-orcid":false,"given":"Eva","family":"Van Braeckel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5609-3726","authenticated-orcid":false,"given":"Stephanie","family":"Van Biervliet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8138-5405","authenticated-orcid":false,"given":"Mieke","family":"Boon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3143-3661","authenticated-orcid":false,"given":"Anabela Santo","family":"Ramalho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Vermeulen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"19","key":"10.1016\/j.jcf.2025.12.018_bib0001","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor\u2013tezacaftor\u2013ivacaftor for cystic fibrosis with a single Phe508del allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N Engl J Med (Baltimore)"},{"issue":"3","key":"10.1016\/j.jcf.2025.12.018_bib0002","first-page":"255","article-title":"From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations","volume":"31","author":"Veit","year":"2020","journal-title":"Mol Biol Cell"},{"key":"10.1016\/j.jcf.2025.12.018_bib0003","doi-asserted-by":"crossref","DOI":"10.1016\/S2213-2600(24)00411-9","article-title":"Vanzacaftor\u2013tezacaftor\u2013deutivacaftor versus elexacaftor\u2013tezacaftor\u2013ivacaftor in individuals with cystic fibrosis aged 12 years and older (SKYLINE Trials VX20-121-102 and VX20-121-103): results from two randomized, active-controlled, phase 3 trials","author":"Keating","year":"2025","journal-title":"Lancet Respir Med (Baltimore)"},{"key":"10.1016\/j.jcf.2025.12.018_bib0004","doi-asserted-by":"crossref","DOI":"10.1016\/S2213-2600(24)00407-7","article-title":"Vanzacaftor\u2013tezacaftor\u2013deutivacaftor for children aged 6\u201311 years with cystic fibrosis (RIDGELINE Trial VX21-121-105): an analysis from a single-arm, phase 3 trial","author":"Hoppe","year":"2025","journal-title":"Lancet Respir Med (Baltimore)"},{"issue":"4","key":"10.1016\/j.jcf.2025.12.018_bib0006","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1016\/j.jcf.2024.02.006","article-title":"In vitro modulator responsiveness of 655 CFTR variants found in people with cystic fibrosis","volume":"23","author":"Bihler","year":"2024","journal-title":"J Cyst Fibros"},{"issue":"11","key":"10.1016\/j.jcf.2025.12.018_bib0007","doi-asserted-by":"crossref","first-page":"888","DOI":"10.1016\/S2213-2600(24)00208-X","article-title":"The expanded French compassionate programme for elexacaftor\u2013tezacaftor\u2013ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study","volume":"12","author":"Burgel","year":"2024","journal-title":"Lancet Respir Med (Baltimore)"},{"key":"10.1016\/j.jcf.2025.12.018_bib0008","unstructured":"The Clinical and Functional TRanslation of CFTR (CFTR2); available at http:\/\/cftr2.org."},{"issue":"1","key":"10.1016\/j.jcf.2025.12.018_bib0009","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1513\/AnnalsATS.201708-668PS","article-title":"The US Food and Drug Administration's experience with ivacaftor in cystic fibrosis. Establishing efficacy using in vitro data in lieu of a clinical trial","volume":"15","author":"Durmowicz","year":"2018","journal-title":"Ann Am Thorac Soc"},{"key":"10.1016\/j.jcf.2025.12.018_bib0010","doi-asserted-by":"crossref","DOI":"10.1016\/j.jcf.2025.02.014","article-title":"The multiple tales on sweat chloride in cystic fibrosis","author":"T\u00fcmmler","year":"2025","journal-title":"J Cyst Fibros"},{"issue":"2","key":"10.1016\/j.jcf.2025.12.018_bib0011","doi-asserted-by":"crossref","first-page":"119","DOI":"10.3390\/cimb47020119","article-title":"Unraveling the mechanism of action, binding sites, and therapeutic advances of CFTR modulators: a narrative review","volume":"47","author":"Baroni","year":"2025","journal-title":"Curr Issues Mol Biol"},{"issue":"1","key":"10.1016\/j.jcf.2025.12.018_bib0012","doi-asserted-by":"crossref","DOI":"10.1016\/j.xpro.2020.100019","article-title":"Protocol for application, standardization, and validation of the forskolin-induced swelling assay in cystic fibrosis human colon organoids","volume":"1","author":"Vonk","year":"2020","journal-title":"STAR Protocols"},{"issue":"1","key":"10.1016\/j.jcf.2025.12.018_bib0013","doi-asserted-by":"crossref","first-page":"6668","DOI":"10.1038\/s41467-024-50641-1","article-title":"Allosteric inhibition of CFTR gating by CFTRinh-172 binding in the pore","volume":"15","author":"Gao","year":"2024","journal-title":"Nat Commun"},{"issue":"5","key":"10.1016\/j.jcf.2025.12.018_bib0014","article-title":"Response to Elexacaftor\/Tezacaftor\/Ivacaftor in people with cystic fibrosis with the N1303K mutation: case report and review of the literature","volume":"10","author":"Ortiz","year":"2024","journal-title":"Heliyon"},{"issue":"11","key":"10.1016\/j.jcf.2025.12.018_bib0016","doi-asserted-by":"crossref","first-page":"1589","DOI":"10.1164\/rccm.202004-1200LE","article-title":"Comparison of organoid swelling and in vivo biomarkers of CFTR function to determine effects of lumacaftor\u2013ivacaftor in patients with cystic fibrosis homozygous for the F508del mutation","volume":"202","author":"Graeber","year":"2020","journal-title":"Am J Respir Crit Care Med"}],"container-title":["Journal of Cystic Fibrosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199325025500?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199325025500?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T11:36:14Z","timestamp":1781782574000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1569199325025500"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3]]},"references-count":14,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,3]]}},"alternative-id":["S1569199325025500"],"URL":"https:\/\/doi.org\/10.1016\/j.jcf.2025.12.018","relation":{},"ISSN":["1569-1993"],"issn-type":[{"value":"1569-1993","type":"print"}],"subject":[],"published":{"date-parts":[[2026,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Rethinking CFTR variant responsiveness: Differential responses to vanzacaftor and elexacaftor","name":"articletitle","label":"Article Title"},{"value":"Journal of Cystic Fibrosis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jcf.2025.12.018","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}]}}