{"id":"https://openalex.org/W7165754452","doi":"https://doi.org/10.3390/ijms27198803","title":"Assessment of CFTR Dysfunction and Responsiveness to CFTR Modulators in COPD Bronchial Air\u2013Liquid Interface Cultures","display_name":"Assessment of CFTR Dysfunction and Responsiveness to CFTR Modulators in COPD Bronchial Air\u2013Liquid Interface Cultures","publication_year":2026,"publication_date":"2026-10-01","ids":{"openalex":"https://openalex.org/W7165754452","doi":"https://doi.org/10.3390/ijms27198803","pmid":"https://pubmed.ncbi.nlm.nih.gov/42884046","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/13664796"},"language":"en","primary_location":{"id":"doi:10.3390/ijms27198803","doi":"https://doi.org/10.3390/ijms27198803","is_oa":true,"landing_page_url":"https://doi.org/10.3390/ijms27198803","pdf_url":null,"source":{"id":"https://openalex.org/S10623703","display_name":"International Journal of Molecular Sciences","issn_l":"1422-0067","issn":["1422-0067","1661-6596"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","medline","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310310987","host_organization_name":"Multidisciplinary Digital Publishing Institute","host_organization_lineage":["https://openalex.org/P4310310987"],"host_organization_lineage_names":["Multidisciplinary Digital Publishing Institute"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"International Journal of Molecular Sciences","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref","pubmed"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://doi.org/10.3390/ijms27198803","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5090625163","display_name":"Nikhil T Awatade","orcid":"https://orcid.org/0000-0003-1560-7991","observed_orcids":["https://orcid.org/0000-0003-1560-7991"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Nikhil T. Awatade","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5035594707","display_name":"Kurtis F. Budden","orcid":"https://orcid.org/0000-0002-7435-275X","observed_orcids":["https://orcid.org/0000-0002-7435-275X"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Kurtis F. Budden","raw_affiliation_strings":[],"raw_orcid":"https://orcid.org/0000-0002-7435-275X","affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5072353038","display_name":"Prabuddha S. Pathinayake","orcid":"https://orcid.org/0000-0002-4947-5918","observed_orcids":["https://orcid.org/0000-0002-4947-5918"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Prabuddha S. Pathinayake","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5026882361","display_name":"Andrew T. Reid","orcid":"https://orcid.org/0000-0001-5751-7025","observed_orcids":["https://orcid.org/0000-0001-5751-7025"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Andrew T. Reid","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"middle","author":{"id":"https://openalex.org/A5038771478","display_name":"Kristy Nichol","orcid":"https://orcid.org/0000-0002-7912-9709","observed_orcids":["https://orcid.org/0000-0002-7912-9709"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Kristy S. Nichol","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"last","author":{"id":"https://openalex.org/A5023800164","display_name":"Peter A. B. Wark","orcid":"https://orcid.org/0000-0001-5676-6126","observed_orcids":["https://orcid.org/0000-0001-5676-6126"]},"institutions":[],"countries":[],"is_corresponding":true,"raw_author_name":"Peter A.B. Wark","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":["https://openalex.org/A5023800164","https://openalex.org/A5090625163"],"corresponding_institution_ids":[],"apc_list":{"value":2900,"currency":"CHF","value_usd":3499},"apc_paid":{"value":2900,"currency":"CHF","value_usd":3499},"fwci":0.0,"has_fulltext":true,"cited_by_count":0,"citation_normalized_percentile":{"value":0.45496014,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"27","issue":"19","first_page":"8803","last_page":"8803"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10665","display_name":"Cystic Fibrosis Research Advances","score":0.9916999936103821,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10665","display_name":"Cystic Fibrosis Research Advances","score":0.9916999936103821,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10143","display_name":"Chronic Obstructive Pulmonary Disease (COPD) Research","score":0.006500000134110451,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11651","display_name":"Inhalation and Respiratory Drug Delivery","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/chronic-obstructive-pulmonary-disease","display_name":"chronic obstructive pulmonary disease","score":0.9089999794960022},{"id":"https://openalex.org/keywords/cftr-dysfunction","display_name":"CFTR dysfunction","score":0.9440000057220459},{"id":"https://openalex.org/keywords/cftr-modulators","display_name":"CFTR modulators","score":0.9700000286102295},{"id":"https://openalex.org/keywords/cigarette-smoke","display_name":"cigarette smoke","score":0.5379999876022339},{"id":"https://openalex.org/keywords/bronchial-epithelial-cells","display_name":"bronchial epithelial cells","score":0.859000027179718},{"id":"https://openalex.org/keywords/air-liquid-interface-culture","display_name":"air-liquid interface culture","score":0.8759999871253967},{"id":"https://openalex.org/keywords/chloride-transport","display_name":"chloride transport","score":0.7919999957084656},{"id":"https://openalex.org/keywords/calcium-activated-chloride-current","display_name":"calcium-activated chloride current","score":0.8769999742507935},{"id":"https://openalex.org/keywords/ion-channels","display_name":"ion channels","score":0.5649999976158142},{"id":"https://openalex.org/keywords/cftr-potentiators","display_name":"CFTR potentiators","score":0.8019999861717224},{"id":"https://openalex.org/keywords/pack-years","display_name":"pack-years","score":0.6830000281333923},{"id":"https://openalex.org/keywords/mucociliary-dysfunction","display_name":"mucociliary dysfunction","score":0.5899999737739563},{"id":"https://openalex.org/keywords/cftr-targeted-therapy","display_name":"CFTR-targeted therapy","score":0.49000000953674316}],"concepts":[{"id":"https://openalex.org/C2778428886","wikidata":"https://www.wikidata.org/wiki/Q420470","display_name":"Cystic fibrosis transmembrane conductance regulator","level":3,"score":0.8061000108718872},{"id":"https://openalex.org/C2776780178","wikidata":"https://www.wikidata.org/wiki/Q199804","display_name":"COPD","level":2,"score":0.8022000193595886},{"id":"https://openalex.org/C2776938444","wikidata":"https://www.wikidata.org/wiki/Q178194","display_name":"Cystic fibrosis","level":2,"score":0.7886000275611877},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.675000011920929},{"id":"https://openalex.org/C114682108","wikidata":"https://www.wikidata.org/wiki/Q1952044","display_name":"Mucociliary clearance","level":3,"score":0.6625999808311462},{"id":"https://openalex.org/C164718446","wikidata":"https://www.wikidata.org/wiki/Q7234839","display_name":"Potentiator","level":2,"score":0.5877000093460083},{"id":"https://openalex.org/C100094513","wikidata":"https://www.wikidata.org/wiki/Q180436","display_name":"Cilium","level":2,"score":0.541100025177002},{"id":"https://openalex.org/C145822097","wikidata":"https://www.wikidata.org/wiki/Q106916978","display_name":"Chloride channel","level":2,"score":0.5353999733924866},{"id":"https://openalex.org/C6507245","wikidata":"https://www.wikidata.org/wiki/Q424948","display_name":"Epithelial sodium channel","level":3,"score":0.5281000137329102},{"id":"https://openalex.org/C87568996","wikidata":"https://www.wikidata.org/wiki/Q1428754","display_name":"Respiratory epithelium","level":3,"score":0.5184000134468079},{"id":"https://openalex.org/C50254741","wikidata":"https://www.wikidata.org/wiki/Q62536","display_name":"Ion channel","level":3,"score":0.4675999879837036},{"id":"https://openalex.org/C127716648","wikidata":"https://www.wikidata.org/wiki/Q104053","display_name":"Phenotype","level":3,"score":0.4611999988555908},{"id":"https://openalex.org/C105922876","wikidata":"https://www.wikidata.org/wiki/Q1423981","display_name":"Airway","level":2,"score":0.45500001311302185},{"id":"https://openalex.org/C529295009","wikidata":"https://www.wikidata.org/wiki/Q41301","display_name":"Epithelium","level":2,"score":0.43380001187324524},{"id":"https://openalex.org/C2776502428","wikidata":"https://www.wikidata.org/wiki/Q6095693","display_name":"Ivacaftor","level":4,"score":0.41200000047683716},{"id":"https://openalex.org/C99975950","wikidata":"https://www.wikidata.org/wiki/Q911011","display_name":"Ion transporter","level":3,"score":0.3725999891757965},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3716000020503998},{"id":"https://openalex.org/C24530287","wikidata":"https://www.wikidata.org/wiki/Q424204","display_name":"Transmembrane protein","level":3,"score":0.3628000020980835},{"id":"https://openalex.org/C79879829","wikidata":"https://www.wikidata.org/wiki/Q5571762","display_name":"Intracellular","level":2,"score":0.3499000072479248},{"id":"https://openalex.org/C28938303","wikidata":"https://www.wikidata.org/wiki/Q17122952","display_name":"Transepithelial potential difference","level":4,"score":0.3345000147819519},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.32350000739097595},{"id":"https://openalex.org/C1491633281","wikidata":"https://www.wikidata.org/wiki/Q7868","display_name":"Cell","level":2,"score":0.3147999942302704},{"id":"https://openalex.org/C25194021","wikidata":"https://www.wikidata.org/wiki/Q17157162","display_name":"Mechanosensation","level":4,"score":0.31349998712539673},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.30889999866485596},{"id":"https://openalex.org/C2776914184","wikidata":"https://www.wikidata.org/wiki/Q101991","display_name":"Inflammation","level":2,"score":0.2939999997615814},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.29269999265670776},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.2847000062465668},{"id":"https://openalex.org/C68324877","wikidata":"https://www.wikidata.org/wiki/Q421859","display_name":"Interleukin 8","level":3,"score":0.27790001034736633},{"id":"https://openalex.org/C2776178081","wikidata":"https://www.wikidata.org/wiki/Q3286546","display_name":"Respiratory disease","level":3,"score":0.27570000290870667},{"id":"https://openalex.org/C105580179","wikidata":"https://www.wikidata.org/wiki/Q188928","display_name":"Messenger RNA","level":3,"score":0.27140000462532043},{"id":"https://openalex.org/C6929976","wikidata":"https://www.wikidata.org/wiki/Q3771881","display_name":"Regulator","level":3,"score":0.26759999990463257},{"id":"https://openalex.org/C534529494","wikidata":"https://www.wikidata.org/wiki/Q7891","display_name":"Respiratory system","level":2,"score":0.26499998569488525},{"id":"https://openalex.org/C2777312293","wikidata":"https://www.wikidata.org/wiki/Q3649349","display_name":"Mucus","level":2,"score":0.2547999918460846}],"mesh":[{"descriptor_ui":"D000627","descriptor_name":"Aminophenols","qualifier_ui":"Q000494","qualifier_name":"pharmacology","is_major_topic":false},{"descriptor_ui":"D001980","descriptor_name":"Bronchi","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":true},{"descriptor_ui":"D001980","descriptor_name":"Bronchi","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":true},{"descriptor_ui":"D001980","descriptor_name":"Bronchi","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true},{"descriptor_ui":"D002478","descriptor_name":"Cells, Cultured","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002923","descriptor_name":"Cilia","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D002923","descriptor_name":"Cilia","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D004847","descriptor_name":"Epithelial Cells","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D004847","descriptor_name":"Epithelial Cells","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D004847","descriptor_name":"Epithelial Cells","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008875","descriptor_name":"Middle Aged","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D015363","descriptor_name":"Quinolones","qualifier_ui":"Q000494","qualifier_name":"pharmacology","is_major_topic":false},{"descriptor_ui":"D019005","descriptor_name":"Cystic Fibrosis Transmembrane Conductance Regulator","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":true},{"descriptor_ui":"D019005","descriptor_name":"Cystic Fibrosis Transmembrane Conductance Regulator","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":true},{"descriptor_ui":"D029424","descriptor_name":"Pulmonary Disease, Chronic Obstructive","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":true},{"descriptor_ui":"D029424","descriptor_name":"Pulmonary Disease, Chronic Obstructive","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true}],"locations_count":4,"locations":[{"id":"doi:10.3390/ijms27198803","doi":"https://doi.org/10.3390/ijms27198803","is_oa":true,"landing_page_url":"https://doi.org/10.3390/ijms27198803","pdf_url":null,"source":{"id":"https://openalex.org/S10623703","display_name":"International Journal of Molecular Sciences","issn_l":"1422-0067","issn":["1422-0067","1661-6596"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","medline","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310310987","host_organization_name":"Multidisciplinary Digital Publishing Institute","host_organization_lineage":["https://openalex.org/P4310310987"],"host_organization_lineage_names":["Multidisciplinary Digital Publishing Institute"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"International Journal of Molecular Sciences","raw_type":"journal-article"},{"id":"doi:10.20944/preprints202606.1673.v1","doi":"https://doi.org/10.20944/preprints202606.1673.v1","is_oa":true,"landing_page_url":"https://doi.org/10.20944/preprints202606.1673.v1","pdf_url":null,"source":{"id":"https://openalex.org/S6309402219","display_name":"Preprints.org","issn_l":null,"issn":null,"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":["https://openalex.org/P4310310987"],"host_organization_lineage_names":["Multidisciplinary Digital Publishing Institute"],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"posted-content"},{"id":"pmh:oai:pubmedcentral.nih.gov:13664796","doi":null,"is_oa":true,"landing_page_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC13664796/","pdf_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC13664796/pdf/ijms-27-08803.pdf","source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Int J Mol Sci","raw_type":"Text"},{"id":"pmid:42884046","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/42884046","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"International journal of molecular sciences","raw_type":"Journal Article"}],"best_oa_location":{"id":"doi:10.3390/ijms27198803","doi":"https://doi.org/10.3390/ijms27198803","is_oa":true,"landing_page_url":"https://doi.org/10.3390/ijms27198803","pdf_url":null,"source":{"id":"https://openalex.org/S10623703","display_name":"International Journal of Molecular Sciences","issn_l":"1422-0067","issn":["1422-0067","1661-6596"],"is_oa":true,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","medline","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310310987","host_organization_name":"Multidisciplinary Digital Publishing Institute","host_organization_lineage":["https://openalex.org/P4310310987"],"host_organization_lineage_names":["Multidisciplinary Digital Publishing Institute"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"International Journal of Molecular Sciences","raw_type":"journal-article"},"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":1.0}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":true,"pdf":true},"content_urls":{"pdf":"https://content.openalex.org/works/W7165754452.pdf","grobid_xml":"https://content.openalex.org/works/W7165754452.grobid-xml"},"referenced_works_count":46,"referenced_works":["https://openalex.org/W1967166731","https://openalex.org/W1969591070","https://openalex.org/W1971088574","https://openalex.org/W1973742535","https://openalex.org/W2005808916","https://openalex.org/W2035325378","https://openalex.org/W2043450272","https://openalex.org/W2054316293","https://openalex.org/W2065160977","https://openalex.org/W2071407356","https://openalex.org/W2091751789","https://openalex.org/W2100186169","https://openalex.org/W2112433605","https://openalex.org/W2146514656","https://openalex.org/W2148602450","https://openalex.org/W2154841232","https://openalex.org/W2158351251","https://openalex.org/W2171253100","https://openalex.org/W2262559919","https://openalex.org/W2294868470","https://openalex.org/W2372958563","https://openalex.org/W2517382167","https://openalex.org/W2604137520","https://openalex.org/W2613303203","https://openalex.org/W2759338004","https://openalex.org/W2885499809","https://openalex.org/W2887886950","https://openalex.org/W2928971587","https://openalex.org/W2974714532","https://openalex.org/W2982296199","https://openalex.org/W2985405196","https://openalex.org/W3000186698","https://openalex.org/W3040970632","https://openalex.org/W3113075930","https://openalex.org/W3195812235","https://openalex.org/W3202369970","https://openalex.org/W4200082190","https://openalex.org/W4205833130","https://openalex.org/W4214673710","https://openalex.org/W4311881860","https://openalex.org/W4313455299","https://openalex.org/W4383314774","https://openalex.org/W4383907554","https://openalex.org/W4390400904","https://openalex.org/W4402793120","https://openalex.org/W7128477308"],"related_works":["https://openalex.org/W4200082190","https://openalex.org/W2517382167","https://openalex.org/W4311881860","https://openalex.org/W2086569637","https://openalex.org/W2022811909","https://openalex.org/W3036202249","https://openalex.org/W2059973562","https://openalex.org/W7017808924","https://openalex.org/W2283531227","https://openalex.org/W2148970730"],"abstract_inverted_index":{"Increasing":[0],"evidence":[1],"suggests":[2],"that":[3,216,256],"acquired":[4,229,250,275],"dysfunction":[5,91,252,277,290],"of":[6,20,53,141],"the":[7,86,145,224,243,264,280],"cystic":[8,40],"fibrosis":[9],"transmembrane":[10],"conductance":[11],"regulator":[12],"(CFTR)":[13],"anion":[14,160],"channel":[15,66,137],"occurs":[16],"as":[17],"a":[18],"result":[19],"cigarette":[21],"smoke":[22],"exposure":[23,210],"in":[24,39,50,70,185,212,228,237,242,291],"chronic":[25],"obstructive":[26],"pulmonary":[27],"disease":[28],"(COPD).":[29],"CFTR-targeted":[30],"therapies":[31],"were":[32,127],"developed":[33],"to":[34,60,84,88],"treat":[35],"genetic":[36,273],"CFTR":[37,62,90,98,173,178,201,230,251,266,276],"defects":[38],"fibrosis,":[41],"but":[42,254],"they":[43],"have":[44],"not":[45,204,221,260],"demonstrated":[46],"consistent":[47,247],"clinical":[48],"efficacy":[49],"small":[51],"trials":[52],"patients":[54],"with":[55,80,168,177,192,198,207,248],"COPD.":[56,292],"Here,":[57],"we":[58],"aimed":[59],"characterize":[61],"and":[63,68,83,121,139,162,182,187,240,274,278],"other":[64],"ion":[65,136,238],"activity":[67],"ciliation":[69,241],"primary":[71,109],"bronchial":[72,110],"epithelial":[73,111,289],"cells":[74,112],"(pBECs)":[75,113],"from":[76,105,115],"COPD":[77,122,148,244],"donors":[78,123],"compared":[79,167,197],"healthy":[81,116],"controls,":[82],"determine":[85],"extent":[87],"which":[89],"can":[92],"be":[93],"rescued":[94],"by":[95,155,263],"clinically":[96],"relevant":[97],"modulators.":[99],"Air\u2013liquid":[100],"interface":[101],"(ALI)":[102],"cultures":[103,149],"generated":[104],"conditionally":[106],"reprogrammed":[107],"(CR)":[108],"obtained":[114],"controls":[117],"(n":[118,124],"=":[119,125],"7)":[120,126],"assessed":[128],"for":[129,223,282],"transepithelial":[130],"electrical":[131],"resistance,":[132],"immunofluorescence,":[133],"cilia":[134],"activity,":[135],"function":[138,202],"expression":[140],"cell-type-associated":[142],"markers":[143],"at":[144],"transcript":[146],"level.":[147],"exhibited":[150],"reduced":[151],"ciliated":[152],"area,":[153],"accompanied":[154],"significantly":[156,205],"decreased":[157],"forskolin/IBMX-stimulated":[158],"CFTR-mediated":[159],"transport":[161,239],"ATP-induced":[163],"calcium-activated":[164],"chloride":[165],"currents":[166],"healthy-control":[169],"cultures,":[170],"despite":[171],"comparable":[172],"mRNA":[174],"expression.":[175],"Treatment":[176],"potentiators":[179],"(VX-770,":[180],"GLPG1837":[181],"icenticaftor)":[183],"resulted":[184],"modest":[186],"highly":[188],"variable":[189],"functional":[190],"responses,":[191],"no":[193],"statistically":[194],"significant":[195],"improvement":[196],"vehicle-treated":[199],"controls.":[200],"was":[203],"associated":[206],"cumulative":[208],"smoking":[209,217],"(pack-years)":[211],"this":[213],"cohort,":[214],"suggesting":[215],"burden":[218],"alone":[219],"may":[220],"account":[222],"observed":[225],"inter-individual":[226],"variability":[227],"dysfunction.":[231],"Collectively,":[232],"these":[233,257],"findings":[234],"demonstrate":[235],"abnormalities":[236,258],"airway":[245],"epithelium":[246],"an":[249],"phenotype,":[253],"indicate":[255],"are":[259],"readily":[261],"reversed":[262],"tested":[265],"potentiators.":[267],"These":[268],"results":[269],"highlight":[270],"differences":[271],"between":[272],"underscore":[279],"need":[281],"alternative":[283],"or":[284],"combinatorial":[285],"therapeutic":[286],"strategies":[287],"targeting":[288]},"counts_by_year":[],"updated_date":"2026-11-09T13:22:03.289127","created_date":"2026-06-25T00:00:00"}
