{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T13:05:08Z","timestamp":1780578308003,"version":"3.54.1"},"reference-count":221,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T00:00:00Z","timestamp":1780531200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) dysfunction is increasingly recognized as a key contributor to a broad spectrum of human diseases beyond classical cystic fibrosis (CF). CFTR is a cAMP-regulated chloride and bicarbonate ion channel expressed in both epithelial and non-epithelial tissues, where it regulates ion homeostasis, mucosal hydration, and cellular signaling. Both inherited CFTR mutations and acquired dysfunction resulting from environmental or inflammatory factors can disrupt these physiological processes and drive disease progression. Current evidence linking CFTR dysregulation to respiratory diseases, such as cystic fibrosis, chronic obstructive pulmonary disease (COPD), asthma, and HIV-associated airway disease, as well as cardiovascular, renal, neurological diseases, and cancer, is comprehensively discussed. Mechanistically, impaired CFTR function promotes oxidative stress, chronic inflammation, epithelial barrier dysfunction, altered mucociliary clearance, and dysregulation of signaling pathways, including NF-\u03baB, TGF-\u03b2, PI3K\/Akt, MAPK, and Wnt\/\u03b2-catenin. In the context of HIV infection and cigarette smoke exposure, CFTR suppression is mediated in part by TGF-\u03b2 signaling and miRNA-dependent mechanisms, resulting in compromised airway defense and increased susceptibility to pulmonary complications. Recent studies further demonstrate that CFTR dysregulation alters the expression of genes involved in fibrosis, inflammation, angiogenesis, and epithelial\u2013mesenchymal transition (EMT). Notably, CFTR may act as either a tumor suppressor or a context-dependent oncogene, depending on tissue type and signaling milieu, highlighting its complex role in cancer biology. Advances in CFTR-targeted therapies, including potentiators, correctors, gene therapy, and combination approaches, have markedly improved outcomes in CF and may offer therapeutic potential for diseases associated with acquired CFTR dysfunction. We summarize the systemic consequences of CFTR dysregulation and the need for further mechanistic and translational research to clarify its role across diverse human diseases.<\/jats:p>","DOI":"10.3390\/cells15111034","type":"journal-article","created":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T11:52:30Z","timestamp":1780573950000},"page":"1034","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Dysfunction in Human Diseases: Molecular Mechanisms and Pathophysiological Implications"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1133-268X","authenticated-orcid":false,"given":"Md. Sohanur","family":"Rahman","sequence":"first","affiliation":[{"name":"Department of Pathology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15213, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-2643-5760","authenticated-orcid":false,"given":"Mohammed","family":"Daira","sequence":"additional","affiliation":[{"name":"Department of Pathology, School of Medicine, University of Pittsburgh, Pittsburgh, PA 15213, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2201307","DOI":"10.1183\/13993003.01307-2022","article-title":"Cystic fibrosis transmembrane conductance regulator in COPD: A role in respiratory epithelium and beyond","volume":"61","author":"Mall","year":"2023","journal-title":"Eur. Respir. J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1111\/jebm.12434","article-title":"ATP-binding cassette (ABC) transporters in cancer: A review of recent updates","volume":"14","author":"Wang","year":"2021","journal-title":"J. Evid. Based Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1369\/0022155414546190","article-title":"Cystic fibrosis transmembrane conductance regulator protein (CFTR) expression in the developing human brain: Comparative immunohistochemical study between patients with normal and mutated CFTR","volume":"62","author":"Marcorelles","year":"2014","journal-title":"J. Histochem. Cytochem."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"5417","DOI":"10.1093\/nar\/19.19.5417","article-title":"Expression of the cystic fibrosis transmembrane conductance regulator gene in cells of non-epithelial origin","volume":"19","author":"Yoshimura","year":"1991","journal-title":"Nucleic Acids Res."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"13266","DOI":"10.1074\/jbc.275.18.13266","article-title":"Epithelial Sodium Channels Regulate Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channels in XenopusOocytes","volume":"275","author":"Jiang","year":"2000","journal-title":"J. Biol. Chem."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1091\/mbc.e14-04-0935","article-title":"From CFTR biology toward combinatorial pharmacotherapy: Expanded classification of cystic fibrosis mutations","volume":"27","author":"Veit","year":"2016","journal-title":"Mol. Biol. Cell"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1002\/ame2.12180","article-title":"A review of cystic fibrosis: Basic and clinical aspects","volume":"4","author":"Chen","year":"2021","journal-title":"Anim. Models Exp. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1038\/nsmb882","article-title":"The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR","volume":"12","author":"Du","year":"2005","journal-title":"Nat. Struct. Mol. Biol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/0014-5793(92)81399-7","article-title":"Altered protein folding may be the molecular basis of most cases of cystic fibrosis","volume":"312","author":"Thomas","year":"1992","journal-title":"FEBS Lett."},{"key":"ref_10","first-page":"227","article-title":"Identification of critical target protein for cystic fibrosis using systems biology network approach","volume":"17","author":"Sahrawat","year":"2013","journal-title":"Int. J. Bioautom."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1164\/rccm.201006-0836OC","article-title":"HIV infection and risk for incident pulmonary diseases in the combination antiretroviral therapy era","volume":"183","author":"Crothers","year":"2011","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"7984","DOI":"10.1038\/s41598-018-26095-z","article-title":"Cigarette smoke promotes HIV infection of primary bronchial epithelium and additively suppresses CFTR function","volume":"8","author":"Chinnapaiyan","year":"2018","journal-title":"Sci. Rep."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1007\/s11904-012-0140-x","article-title":"Pathogenesis of HIV and the lung","volume":"10","author":"Gingo","year":"2013","journal-title":"Curr. HIV AIDS Rep."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Chinnapaiyan, S., and Unwalla, H.J. (2015). Mucociliary dysfunction in HIV and smoked substance abuse. Front. Microbiol., 6.","DOI":"10.3389\/fmicb.2015.01052"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1165\/rcmb.2013-0538OC","article-title":"Transforming growth factor-\u03b21 and cigarette smoke inhibit the ability of \u03b22-agonists to enhance epithelial permeability","volume":"52","author":"Unwalla","year":"2015","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1093\/toxsci\/kfq101","article-title":"Cadmium regulates the expression of the CFTR chloride channel in human airway epithelial cells","volume":"116","author":"Rennolds","year":"2010","journal-title":"Toxicol. Sci."},{"key":"ref_17","doi-asserted-by":"crossref","unstructured":"Mitash, N., Mu, F., Donovan, J.E., Myerburg, M.M., Ranganathan, S., Greene, C.M., and Swiatecka-Urban, A. (2019). Transforming Growth Factor-\u03b21 Selectively Recruits microRNAs to the RNA-Induced Silencing Complex and Degrades CFTR mRNA under Permissive Conditions in Human Bronchial Epithelial Cells. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20194933"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"7671","DOI":"10.1074\/jbc.M113.545137","article-title":"Cigarette smoke-induced Ca2+ release leads to cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction","volume":"289","author":"Rasmussen","year":"2014","journal-title":"J. Biol. Chem."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"112090","DOI":"10.1016\/j.biopha.2021.112090","article-title":"Gene-specific MicroRNA antagonism protects against HIV Tat and TGF-\u03b2-mediated suppression of CFTR mRNA and function","volume":"142","author":"Dutta","year":"2021","journal-title":"Biomed. Pharmacother."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1434","DOI":"10.1016\/j.freeradbiomed.2004.03.010","article-title":"Cadmium inhibits the electron transfer chain and induces reactive oxygen species","volume":"36","author":"Wang","year":"2004","journal-title":"Free Radic. Biol. Med."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/j.ncrna.2024.11.001","article-title":"Dr. Jekyll or Mr. Hyde: The multifaceted roles of miR-145-5p in human health and disease","volume":"11","author":"Rahman","year":"2025","journal-title":"Noncoding RNA Res."},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Santiago, M.J., Chinnapaiyan, S., Panda, K., Rahman, M.S., Ghorai, S., Rahman, I., Black, S.M., Liu, Y., and Unwalla, H.J. (2024). Altered Host microRNAomics in HIV Infections: Therapeutic Potentials and Limitations. Int. J. Mol. Sci., 25.","DOI":"10.3390\/ijms25168809"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1023\/A:1020229923095","article-title":"Cadmium biomonitoring and renal dysfunction among a population environmentally exposed to cadmium from smelting in China (ChinaCad)","volume":"15","author":"Jin","year":"2002","journal-title":"Biometals"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/S0140-6736(88)91275-5","article-title":"Cadmium in Lung Tissue as Marker for Smoking","volume":"331","author":"Anttila","year":"1988","journal-title":"Lancet"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1165\/ajrcmb.19.3.3242","article-title":"Cadmium inhibits proteoglycan and procollagen production by cultured human lung fibroblasts","volume":"19","author":"Chambers","year":"1998","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_26","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":"Premchandar","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.jcf.2021.04.016","article-title":"Cardiovascular complications in cystic fibrosis: A review of the literature","volume":"21","author":"Poore","year":"2022","journal-title":"J. Cyst. Fibros."},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"Parisi, G.F., Terlizzi, V., Manti, S., Papale, M., Pecora, G., Presti, S., Tosto, M., and Leonardi, S. (2025). Cutting-Edge Advances in Cystic Fibrosis: From Gene Therapy to Personalized Medicine and Holistic Management. Genes, 16.","DOI":"10.3390\/genes16040402"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1016\/j.chest.2021.09.003","article-title":"Cancer in Cystic Fibrosis: A Narrative Review of Prevalence, Risk Factors, Screening, and Treatment Challenges: Adult Cystic Fibrosis Series","volume":"161","author":"Maisonneuve","year":"2022","journal-title":"Chest"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"107499","DOI":"10.1016\/j.rmed.2023.107499","article-title":"Potential systemic effects of acquired CFTR dysfunction in COPD","volume":"221","author":"Miravitlles","year":"2024","journal-title":"Respir. Med."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1186\/1465-9921-10-23","article-title":"Mechanisms of the noxious inflammatory cycle in cystic fibrosis","volume":"10","author":"Rottner","year":"2009","journal-title":"Respir. Res."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1080\/15476286.2023.2289709","article-title":"Structural and computational studies of HIV-1 RNA","volume":"21","author":"Levintov","year":"2024","journal-title":"RNA Biol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"796","DOI":"10.1093\/nar\/gki220","article-title":"HIV-1 can escape from RNA interference by evolving an alternative structure in its RNA genome","volume":"33","author":"Westerhout","year":"2005","journal-title":"Nucleic Acids Res."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1146\/annurev.med.60.041807.123549","article-title":"Emerging concepts in the immunopathogenesis of AIDS","volume":"60","author":"Douek","year":"2009","journal-title":"Annu. Rev. Med."},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"Caputo, V., Libera, M., Sisti, S., Giuliani, B., Diotti, R.A., and Criscuolo, E. (2023). The initial interplay between HIV and mucosal innate immunity. Front. Immunol., 14.","DOI":"10.3389\/fimmu.2023.1104423"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"12552","DOI":"10.1038\/s41598-019-49056-6","article-title":"TGF-\u03b21 increases viral burden and promotes HIV-1 latency in primary differentiated human bronchial epithelial cells","volume":"9","author":"Chinnapaiyan","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1016\/S0165-2478(02)00023-8","article-title":"Mechanisms of TGF-beta signaling in regulation of cell growth and differentiation","volume":"82","author":"Moustakas","year":"2002","journal-title":"Immunol. Lett."},{"key":"ref_38","first-page":"1","article-title":"HIV Tat Protein Induces the TGF-\u03b2 Signaling Pathway and Suppresses the CFTR Biogenesis and Activity by microRNA-Regulated Gene-Silencing Mechanism","volume":"34","author":"Dutta","year":"2020","journal-title":"FASEB J."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1186\/s12950-024-00405-x","article-title":"MicroRNA mediated suppression of airway lactoperoxidase by TGF-\u03b21 and cigarette smoke promotes airway inflammation","volume":"21","author":"Santiago","year":"2024","journal-title":"J. Inflamm."},{"key":"ref_40","unstructured":"Dutta, R., Chinnapaiyan, S., Nair, M., and Unwalla, H. (2018). HIV Tat Protein and TGF-\u03b2 Suppresses CFTR Biogenesis and Activity by Microrna Mediated Gene Silencing. D53. off the Beaten Path: New Approaches to Copd Pathogenesis, American Thoracic Society."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1016\/j.yexcr.2004.04.026","article-title":"TGF\u03b2 down-regulation of the CFTR: A means to limit epithelial chloride secretion","volume":"298","author":"Howe","year":"2004","journal-title":"Exp. Cell Res."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1325","DOI":"10.1097\/QAD.0b013e328352adca","article-title":"The role of microRNAs in HIV-1 pathogenesis and therapy","volume":"26","author":"Swaminathan","year":"2012","journal-title":"Aids"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1016\/j.ymthe.2018.11.017","article-title":"A neutralizing aptamer to TGFBR2 and miR-145 antagonism rescue cigarette smoke-and TGF-\u03b2-mediated CFTR expression","volume":"27","author":"Dutta","year":"2019","journal-title":"Mol. Ther."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1016\/j.chest.2018.10.006","article-title":"The Clinical Biology of Cystic Fibrosis Transmembrane Regulator Protein: Its Role and Function in Extrapulmonary Disease","volume":"155","author":"Liou","year":"2019","journal-title":"Chest"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"190068","DOI":"10.1183\/16000617.0068-2019","article-title":"CFTR targeted therapies: Recent advances in cystic fibrosis and possibilities in other diseases of the airways","volume":"29","author":"Patel","year":"2020","journal-title":"Eur. Respir. Rev."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"Fso57","DOI":"10.4155\/fso.15.57","article-title":"Cystic fibrosis\u2014A multiorgan protein misfolding disease","volume":"1","year":"2015","journal-title":"Future Sci. OA"},{"key":"ref_47","doi-asserted-by":"crossref","unstructured":"Parisi, G.F., Papale, M., Pecora, G., Rotolo, N., Manti, S., Russo, G., and Leonardi, S. (2023). Cystic Fibrosis and Cancer: Unraveling the Complex Role of CFTR Gene in Cancer Susceptibility. Cancers, 15.","DOI":"10.3390\/cancers15174244"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1164\/rccm.201210-1843CI","article-title":"Chronic bronchitis and chronic obstructive pulmonary disease","volume":"187","author":"Kim","year":"2013","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1513\/pats.201006-045WR","article-title":"HIV and chronic obstructive pulmonary disease: Is it worse and why?","volume":"8","author":"Morris","year":"2011","journal-title":"Proc. Am. Thorac. Soc."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1016\/j.jaci.2011.11.015","article-title":"Asthma diagnosis and airway bronchodilator response in HIV-infected patients","volume":"129","author":"Gingo","year":"2012","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1186\/s12931-015-0294-3","article-title":"Roflumilast partially reverses smoke-induced mucociliary dysfunction","volume":"16","author":"Schmid","year":"2015","journal-title":"Respir. Res."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1186\/s12931-017-0656-0","article-title":"Roflumilast reverses CFTR-mediated ion transport dysfunction in cigarette smoke-exposed mice","volume":"18","author":"Raju","year":"2017","journal-title":"Respir. Res."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1096\/fj.11-192377","article-title":"Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration","volume":"26","author":"Clunes","year":"2012","journal-title":"FASEB J."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"S169","DOI":"10.1513\/AnnalsATS.201503-126AW","article-title":"Immune Dysfunction in Patients with Chronic Obstructive Pulmonary Disease","volume":"12","author":"Bhat","year":"2015","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_55","doi-asserted-by":"crossref","unstructured":"Keown, K., Brown, R., Doherty, D.F., Houston, C., McKelvey, M.C., Creane, S., Linden, D., McAuley, D.F., Kidney, J.C., and Weldon, S. (2020). Airway Inflammation and Host Responses in the Era of CFTR Modulators. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21176379"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"L591","DOI":"10.1152\/ajplung.00335.2013","article-title":"Mechanisms of phagocytosis and host clearance of Pseudomonas aeruginosa","volume":"306","author":"Lovewell","year":"2014","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1164\/rccm.201906-1190PP","article-title":"Targeting Proteases in Cystic Fibrosis Lung Disease. Paradigms, Progress, and Potential","volume":"201","author":"McKelvey","year":"2020","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"L690","DOI":"10.1152\/ajplung.00090.2019","article-title":"Characterization of cystic fibrosis airway smooth muscle cell proliferative and contractile activities","volume":"317","author":"Jang","year":"2019","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1186\/s12967-018-1635-z","article-title":"Roles of airway smooth muscle dysfunction in chronic obstructive pulmonary disease","volume":"16","author":"Yan","year":"2018","journal-title":"J. Transl. Med."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1186\/1465-9921-15-69","article-title":"Accumulation of metals in GOLD4 COPD lungs is associated with decreased CFTR levels","volume":"15","author":"Hassan","year":"2014","journal-title":"Respir. Res."},{"key":"ref_61","doi-asserted-by":"crossref","unstructured":"Li, J., and Ye, Z. (2020). The Potential Role and Regulatory Mechanisms of MUC5AC in Chronic Obstructive Pulmonary Disease. Molecules, 25.","DOI":"10.20944\/preprints202008.0260.v1"},{"key":"ref_62","doi-asserted-by":"crossref","unstructured":"Panda, K., Santiago, M.J., Rahman, M.S., Ghorai, S., Black, S.M., Rahman, I., Unwalla, H.J., and Chinnapaiyan, S. (2024). HIV-1 Tat Protein and Cigarette Smoke Mediated ADAM17 Upregulation Can Lead to Impaired Mucociliary Clearance. Cells, 13.","DOI":"10.3390\/cells13232009"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"717","DOI":"10.1016\/j.cyto.2011.09.010","article-title":"Increased interleukin (IL)-8 and decreased IL-17 production in chronic obstructive pulmonary disease (COPD) provoked by cigarette smoke","volume":"56","author":"He","year":"2011","journal-title":"Cytokine"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1183\/09031936.00036707","article-title":"Increased activation of p38 MAPK in COPD","volume":"31","author":"Renda","year":"2008","journal-title":"Eur. Respir. J."},{"key":"ref_65","doi-asserted-by":"crossref","unstructured":"Christopoulou, M.E., Papakonstantinou, E., and Stolz, D. (2023). Matrix Metalloproteinases in Chronic Obstructive Pulmonary Disease. Int. J. Mol. Sci., 24.","DOI":"10.3390\/ijms24043786"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1016\/j.cellsig.2018.10.002","article-title":"Amphiregulin potentiates airway inflammation and mucus hypersecretion induced by urban particulate matter via the EGFR-PI3K\u03b1-AKT\/ERK pathway","volume":"53","author":"Wang","year":"2019","journal-title":"Cell Signal"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1385\/CBB:47:1:131","article-title":"Molecular mechanisms in chronic obstructive pulmonary disease","volume":"47","author":"Alagappan","year":"2007","journal-title":"Cell Biochem. Biophys."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1378\/chest.09-1029","article-title":"Altered surfactant protein-A expression in type II pneumocytes in COPD","volume":"137","author":"Vlachaki","year":"2010","journal-title":"Chest"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1183\/23120541.00378-2022","article-title":"Multiomics links global surfactant dysregulation with airflow obstruction and emphysema in COPD","volume":"9","author":"Hristova","year":"2023","journal-title":"ERJ Open Res."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1038\/nri1528","article-title":"Immunoregulatory functions of surfactant proteins","volume":"5","author":"Wright","year":"2005","journal-title":"Nat. Rev. Immunol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"3637","DOI":"10.1007\/s00018-015-1946-7","article-title":"Airway hydration and COPD","volume":"72","author":"Ghosh","year":"2015","journal-title":"Cell. Mol. Life Sci."},{"key":"ref_72","doi-asserted-by":"crossref","unstructured":"Wu, M., and Chen, J.-H. (2024). CFTR dysfunction leads to defective bacterial eradication on cystic fibrosis airways. Front. Physiol., 15.","DOI":"10.3389\/fphys.2024.1385661"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1186\/1465-9921-6-151","article-title":"MMP-9, TIMP-1 and inflammatory cells in sputum from COPD patients during exacerbation","volume":"6","author":"Mercer","year":"2005","journal-title":"Respir. Res."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1039\/b924455g","article-title":"CFTR chloride channel in the apical compartments: Spatiotemporal coupling to its interacting partners","volume":"2","author":"Li","year":"2010","journal-title":"Integr. Biol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1016\/j.jcf.2016.06.001","article-title":"Risk of asthma in heterozygous carriers for cystic fibrosis: A meta-analysis","volume":"15","author":"Nielsen","year":"2016","journal-title":"J. Cyst. Fibros."},{"key":"ref_76","unstructured":"Aldossary, A.M. (2018). Correction of the \u0394F508 Mutation in the CFTR Gene by CRISPR\/Cas9 System. [Doctoral Dissertation, UCL (University College London)]."},{"key":"ref_77","doi-asserted-by":"crossref","unstructured":"Hanssens, L.S., Duchateau, J., and Casimir, G.J. (2021). CFTR Protein: Not Just a Chloride Channel?. Cells, 10.","DOI":"10.3390\/cells10112844"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1186\/s12950-015-0107-y","article-title":"CFTR Knockdown induces proinflammatory changes in intestinal epithelial cells","volume":"12","author":"Crites","year":"2015","journal-title":"J. Inflamm."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"51","DOI":"10.15586\/aei.v52i5.1148","article-title":"Allergo-immunopathology mechanism of thymol-inhibiting airway remodeling in asthmatic mice by regulating TGF-\u03b2\/Smad3 pathway","volume":"52","author":"Zhang","year":"2024","journal-title":"Allergol. Immunopathol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1124\/jpet.120.000150","article-title":"Angiotensin-(1-7) rescues chronic intermittent hypoxia-aggravated TGF-\u03b2-mediated airway remodeling in murine and cellular models of asthma","volume":"375","author":"Zhou","year":"2020","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"2589","DOI":"10.1111\/all.16242","article-title":"Therapeutic relevance of eosinophilic inflammation and airway viral interactions in severe asthma","volume":"79","author":"Rupani","year":"2024","journal-title":"Allergy"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"10404","DOI":"10.1038\/s41598-024-60864-3","article-title":"Ratio of plasma IL-13\/TNF-\u2009\u221d\u2009and CXCL10\/CCL17 predicts mepolizumab and omalizumab response in asthma better than eosinophil count or immunoglobulin E level","volume":"14","author":"Akenroye","year":"2024","journal-title":"Sci. Rep."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1172\/JCI5909","article-title":"Pulmonary expression of interleukin-13 causes inflammation, mucus hypersecretion, subepithelial fibrosis, physiologic abnormalities, and eotaxin production","volume":"103","author":"Zhu","year":"1999","journal-title":"J. Clin. Investig."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.cmet.2011.04.013","article-title":"Protection from obesity and diabetes by blockade of TGF-\u03b2\/Smad3 signaling","volume":"14","author":"Yadav","year":"2011","journal-title":"Cell Metab."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"1977","DOI":"10.1113\/jphysiol.2007.149310","article-title":"Munc13-2-\/- baseline secretion defect reveals source of oligomeric mucins in mouse airways","volume":"586","author":"Zhu","year":"2008","journal-title":"J. Physiol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"L414","DOI":"10.1152\/ajplung.00346.2010","article-title":"Epithelial EGF receptor signaling mediates airway hyperreactivity and remodeling in a mouse model of chronic asthma","volume":"300","author":"Acciani","year":"2011","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_87","doi-asserted-by":"crossref","unstructured":"Koumpagioti, D., Moriki, D., Boutopoulou, B., Matziou, V., Loukou, I., Priftis, K.N., and Douros, K. (2023). The Association between CFTR Gene Mutation Heterozygosity and Asthma Development: A Systematic Review. J. Clin. Med., 12.","DOI":"10.3390\/jcm12062403"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1016\/S0140-6736(77)91814-1","article-title":"Cystic-fibrosis heterozygosity in childhood bronchial asthma","volume":"1","author":"Gyurkovits","year":"1977","journal-title":"Lancet"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"4485","DOI":"10.1007\/s00018-020-03540-9","article-title":"Dysregulated signalling pathways in innate immune cells with cystic fibrosis mutations","volume":"77","author":"Holbrook","year":"2020","journal-title":"Cell. Mol. Life Sci."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"1404","DOI":"10.1016\/j.jaci.2012.07.023","article-title":"Phenotypic characterization of lung macrophages in asthmatic patients: Overexpression of CCL17","volume":"130","author":"Staples","year":"2012","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1513\/pats.200703-044BR","article-title":"Sputum biomarkers of inflammation in cystic fibrosis lung disease","volume":"4","author":"Sagel","year":"2007","journal-title":"Proc. Am. Thorac. Soc."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"5086","DOI":"10.1111\/febs.70050","article-title":"Inflammation and epithelial-mesenchymal transition in a CFTR-depleted human bronchial epithelial cell line revealed by proteomics and human organ-on-a-chip","volume":"292","author":"Mattoscio","year":"2025","journal-title":"FEBS J."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1165\/rcmb.2010-0224OC","article-title":"Lack of cystic fibrosis transmembrane conductance regulator in CD3+ lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses","volume":"44","author":"Mueller","year":"2011","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"e159832","DOI":"10.1172\/jci.insight.159832","article-title":"IL-13\u2013programmed airway tuft cells produce PGE2, which promotes CFTR-dependent mucociliary function","volume":"7","author":"Kotas","year":"2022","journal-title":"JCI Insight"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1186\/s12931-023-02495-2","article-title":"CFTR dysfunction in smooth muscle drives TGF\u03b2 dependent airway hyperreactivity","volume":"24","author":"Kramer","year":"2023","journal-title":"Respir. Res."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1203\/PDR.0b013e318204edb5","article-title":"Effects of IL-13 on Mucociliary Differentiation of Pediatric Asthmatic Bronchial Epithelial Cells","volume":"69","author":"Thavagnanam","year":"2011","journal-title":"Pediatr. Res."},{"key":"ref_97","doi-asserted-by":"crossref","unstructured":"Kim, S., Beyer, B.A., Lewis, C., and Nadel, J.A. (2013). Normal CFTR inhibits epidermal growth factor receptor-dependent pro-inflammatory chemokine production in human airway epithelial cells. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0072981"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"C700","DOI":"10.1152\/ajpcell.1999.276.3.C700","article-title":"Chemokine expression in CF epithelia: Implications for the role of CFTR in RANTES expression","volume":"276","author":"Schwiebert","year":"1999","journal-title":"Am. J. Physiol."},{"key":"ref_99","doi-asserted-by":"crossref","unstructured":"Amaral, M.D., Quaresma, M.C., and Pankonien, I. (2020). What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer?. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21093133"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"3416","DOI":"10.1039\/D5MD00203F","article-title":"The relationship between cancer risk and cystic fibrosis: The role of CFTR in cell growth and cancer development","volume":"16","author":"Indra","year":"2025","journal-title":"RSC Med. Chem."},{"key":"ref_101","doi-asserted-by":"crossref","unstructured":"Scott, P., Anderson, K., Singhania, M., and Cormier, R. (2020). Cystic Fibrosis, CFTR, and Colorectal Cancer. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21082891"},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"920","DOI":"10.1038\/s41419-020-03119-z","article-title":"Mutant CFTR Drives TWIST1 mediated epithelial\u2013mesenchymal transition","volume":"11","author":"Quaresma","year":"2020","journal-title":"Cell Death Dis."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"4191","DOI":"10.1038\/onc.2015.483","article-title":"CFTR is a tumor suppressor gene in murine and human intestinal cancer","volume":"35","author":"Than","year":"2016","journal-title":"Oncogene"},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1002\/ijc.24394","article-title":"Methylation of multiple genes as molecular markers for diagnosis of a small, well-differentiated hepatocellular carcinoma","volume":"125","author":"Moribe","year":"2009","journal-title":"Int. J. Cancer"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1186\/s13148-018-0496-x","article-title":"Urine cell-based DNA methylation classifier for monitoring bladder cancer","volume":"10","author":"Mengual","year":"2018","journal-title":"Clin. Epigenet."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"2028","DOI":"10.1016\/j.ajpath.2013.02.040","article-title":"Genome-wide methylation analysis of prostate tissues reveals global methylation patterns of prostate cancer","volume":"182","author":"Luo","year":"2013","journal-title":"Am. J. Pathol."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"1203","DOI":"10.1111\/j.1440-1843.2011.01994.x","article-title":"Promoter hypermethylation of the CFTR gene and clinical\/pathological features associated with non-small cell lung cancer","volume":"16","author":"Son","year":"2011","journal-title":"Respirology"},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1002\/cbin.11260","article-title":"Promoter hypermethylation of the CFTR gene as a novel diagnostic and prognostic marker of breast cancer","volume":"44","author":"Liu","year":"2020","journal-title":"Cell Biol. Int."},{"key":"ref_109","doi-asserted-by":"crossref","unstructured":"Shin, Y., Kim, M., Won, J., Kim, J., Oh, S.B., Lee, J.H., and Park, K. (2020). Epigenetic Modification of CFTR in Head and Neck Cancer. J. Clin. Med., 9.","DOI":"10.3390\/jcm9030734"},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"7301","DOI":"10.1111\/jcmm.15300","article-title":"CFTR promotes malignant glioma development via up-regulation of Akt\/Bcl2-mediated anti-apoptosis pathway","volume":"24","author":"Zhao","year":"2020","journal-title":"J. Cell. Mol. Med."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"2227","DOI":"10.3892\/or.2015.3829","article-title":"High level of CFTR expression is associated with tumor aggression and knockdown of CFTR suppresses proliferation of ovarian cancer in vitro and in vivo","volume":"33","author":"Xu","year":"2015","journal-title":"Oncol. Rep."},{"key":"ref_112","doi-asserted-by":"crossref","unstructured":"Bhattacharya, R., Blankenheim, Z., Scott, P.M., and Cormier, R.T. (2022). CFTR and Gastrointestinal Cancers: An Update. J. Pers. Med., 12.","DOI":"10.3390\/jpm12060868"},{"key":"ref_113","doi-asserted-by":"crossref","unstructured":"Santarpia, M., Aguilar, A., Chaib, I., Cardona, A.F., Fancelli, S., Laguia, F., Bracht, J.W.P., Cao, P., Molina-Vila, M.A., and Karachaliou, N. (2020). Non-Small-Cell Lung Cancer Signaling Pathways, Metabolism, and PD-1\/PD-L1 Antibodies. Cancers, 12.","DOI":"10.3390\/cancers12061475"},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"1737","DOI":"10.1007\/s00018-018-2755-6","article-title":"Cystic fibrosis transmembrane conductance regulator-emerging regulator of cancer","volume":"75","author":"Zhang","year":"2018","journal-title":"Cell. Mol. Life Sci."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1007\/s00432-024-05714-5","article-title":"Regulation of VEGF-A expression and VEGF-A-targeted therapy in malignant tumors","volume":"150","author":"Kang","year":"2024","journal-title":"J. Cancer Res. Clin. Oncol."},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"2759","DOI":"10.1002\/jcp.23020","article-title":"CFTR negatively regulates cyclooxygenase-2-PGE(2) positive feedback loop in inflammation","volume":"227","author":"Chen","year":"2012","journal-title":"J. Cell. Physiol."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.4251\/wjgo.v13.i9.1121","article-title":"Screening strategy for gastrointestinal and hepatopancreatobiliary cancers in cystic fibrosis","volume":"13","author":"Hoskins","year":"2021","journal-title":"World J. Gastrointest. Oncol."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"S453","DOI":"10.14309\/01.ajg.0001135904.55345.c8","article-title":"S2111 CFTR Modulators Are Not Associated With Reduction in Cancer Incidence: A Propensity Matched Analysis of 15,280 Cystic Fibrosis Patients","volume":"120","author":"Yarra","year":"2025","journal-title":"Off. J. Am. Coll. Gastroenterol. ACG"},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"2961","DOI":"10.1016\/j.bbamcr.2013.07.021","article-title":"Downregulation of CFTR promotes epithelial-to-mesenchymal transition and is associated with poor prognosis of breast cancer","volume":"1833","author":"Zhang","year":"2013","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Cell Res."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"17239","DOI":"10.1074\/jbc.M112456200","article-title":"Tyrosine Kinase c-Src Constitutes a Bridge between Cystic Fibrosis Transmembrane Regulator Channel Failure and MUC1 Overexpression in Cystic Fibrosis","volume":"277","author":"Cafferata","year":"2002","journal-title":"J. Biol. Chem."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"1553","DOI":"10.1183\/09031936.00164212","article-title":"CFTR dysfunction induces vascular endothelial growth factor synthesis in airway epithelium","volume":"42","author":"Martin","year":"2013","journal-title":"Eur. Respir. J."},{"key":"ref_122","doi-asserted-by":"crossref","unstructured":"Ramananda, Y., Naren, A.P., and Arora, K. (2024). Functional Consequences of CFTR Interactions in Cystic Fibrosis. Int. J. Mol. Sci., 25.","DOI":"10.3390\/ijms25063384"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1152\/physiol.00024.2023","article-title":"Pulmonary Vascular Dysfunctions in Cystic Fibrosis","volume":"39","author":"Amoakon","year":"2024","journal-title":"Physiology"},{"key":"ref_124","first-page":"e16723","article-title":"Cardiovascular System Involvement in Cystic Fibrosis","volume":"13","author":"Shah","year":"2021","journal-title":"Cureus"},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"2101861","DOI":"10.1183\/13993003.01861-2021","article-title":"CFTR in the regulation of pulmonary vascular tone and remodeling","volume":"58","author":"Tabeling","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"914","DOI":"10.1097\/FJC.0000000000001257","article-title":"CFTR Suppresses Neointimal Formation Through Attenuating Proliferation and Migration of Aortic Smooth Muscle Cells","volume":"79","author":"Lu","year":"2022","journal-title":"J. Cardiovasc. Pharmacol."},{"key":"ref_127","doi-asserted-by":"crossref","unstructured":"Kolur, V., Vastrad, B., Vastrad, C., Kotturshetti, S., and Tengli, A. (2021). Identification of candidate biomarkers and therapeutic agents for heart failure by bioinformatics analysis. BMC Cardiovasc. Disord., 21.","DOI":"10.1186\/s12872-021-02146-8"},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"940","DOI":"10.1016\/j.jacbts.2019.07.004","article-title":"CFTR Therapeutics Normalize Cerebral Perfusion Deficits in Mouse Models of Heart Failure and Subarachnoid Hemorrhage","volume":"4","author":"Lidington","year":"2019","journal-title":"JACC Basic Transl. Sci."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1016\/j.jcf.2012.11.012","article-title":"Left ventricular and aortic dysfunction in cystic fibrosis mice","volume":"12","author":"Sellers","year":"2013","journal-title":"J. Cyst. Fibros."},{"key":"ref_130","doi-asserted-by":"crossref","unstructured":"Lee, J., Lee, S., Zhang, H., Hill, M.A., Zhang, C., and Park, Y. (2017). Interaction of IL-6 and TNF-\u03b1 contributes to endothelial dysfunction in type 2 diabetic mouse hearts. PLoS ONE, 12.","DOI":"10.1371\/journal.pone.0187189"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"2000261","DOI":"10.1183\/13993003.00261-2020","article-title":"Transcriptomic analysis of CFTR-impaired endothelial cells reveals a pro-inflammatory phenotype","volume":"57","author":"Declercq","year":"2021","journal-title":"Eur. Respir. J."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1161\/ATVBAHA.120.314622","article-title":"Surfactant protein A, a novel regulator for smooth muscle phenotypic modulation and vascular remodeling\u2014Brief report","volume":"41","author":"Ran","year":"2021","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"H1694","DOI":"10.1152\/ajpheart.00908.2008","article-title":"IL-6 loss causes ventricular dysfunction, fibrosis, reduced capillary density, and dramatically alters the cell populations of the developing and adult heart","volume":"296","author":"Banerjee","year":"2009","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"ref_134","first-page":"2954","article-title":"TNF-alpha drives remodeling of blood vessels and lymphatics in sustained airway inflammation in mice","volume":"119","author":"Baluk","year":"2009","journal-title":"J. Clin. Investig."},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1038\/labinvest.2016.65","article-title":"Hypoxia-stimulated cardiac fibroblast production of IL-6 promotes myocardial fibrosis via the TGF-\u03b21 signaling pathway","volume":"96","author":"Wang","year":"2016","journal-title":"Lab. Investig."},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"552","DOI":"10.1136\/thx.2006.060293","article-title":"COX-2: A link between airway inflammation and disordered chloride secretion in cystic fibrosis?","volume":"61","author":"Clayton","year":"2006","journal-title":"Thorax"},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.cardiores.2004.05.011","article-title":"IL-6 plays an obligatory role in late preconditioning via JAK-STAT signaling and upregulation of iNOS and COX-2","volume":"64","author":"Dawn","year":"2004","journal-title":"Cardiovasc. Res."},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1590\/S0001-37652000000300013","article-title":"The cystic fibrosis transmembrane regulator (CFTR) in the kidney","volume":"72","author":"Morales","year":"2000","journal-title":"An. Acad. Bras. Cienc."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"sfae256","DOI":"10.1093\/ckj\/sfae256","article-title":"Kidney effects of triple CFTR modulator therapy in people with cystic fibrosis","volume":"17","author":"Gabai","year":"2024","journal-title":"Clin. Kidney J."},{"key":"ref_140","doi-asserted-by":"crossref","first-page":"3605","DOI":"10.1007\/s00467-025-06715-3","article-title":"Cystic fibrosis-related kidney disease-emerging morbidity and disease modifier","volume":"40","author":"Hart","year":"2025","journal-title":"Pediatr. Nephrol."},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1016\/j.jcf.2013.03.005","article-title":"A review of renal disease in cystic fibrosis","volume":"12","author":"Nazareth","year":"2013","journal-title":"J. Cyst. Fibros."},{"key":"ref_142","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1159\/000526051","article-title":"Regulation of Distal Nephron Transport by Intracellular Chloride and Potassium","volume":"147","author":"Rodan","year":"2023","journal-title":"Nephron"},{"key":"ref_143","doi-asserted-by":"crossref","first-page":"5233","DOI":"10.1038\/s41598-017-05435-5","article-title":"Defective CFTR leads to aberrant \u03b2-catenin activation and kidney fibrosis","volume":"7","author":"Zhang","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_144","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1038\/kisup.2014.16","article-title":"Wnt\/\u03b2-catenin signaling and kidney fibrosis","volume":"4","author":"Tan","year":"2014","journal-title":"Kidney Int. Suppl."},{"key":"ref_145","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1038\/nrneph.2016.48","article-title":"TGF-\u03b2: The master regulator of fibrosis","volume":"12","author":"Meng","year":"2016","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_146","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1002\/path.5798","article-title":"Tubule-derived INHBB promotes interstitial fibroblast activation and renal fibrosis","volume":"256","author":"Sun","year":"2022","journal-title":"J. Pathol."},{"key":"ref_147","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1085\/jgp.200810097","article-title":"SLC26A9 is a constitutively active, CFTR-regulated anion conductance in human bronchial epithelia","volume":"133","author":"Bertrand","year":"2009","journal-title":"J. Gen. Physiol."},{"key":"ref_148","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1113\/jphysiol.2007.135855","article-title":"SLC26A9 is a Cl(-) channel regulated by the WNK kinases","volume":"584","author":"Dorwart","year":"2007","journal-title":"J. Physiol."},{"key":"ref_149","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.bbrc.2022.06.008","article-title":"HIF-1\u03b1 mediates renal fibrosis by regulating metabolic remodeling of renal tubule epithelial cells","volume":"618","author":"Liu","year":"2022","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_150","doi-asserted-by":"crossref","first-page":"2623","DOI":"10.1007\/s11255-024-03989-8","article-title":"The role of PI3K\/Akt signaling pathway in chronic kidney disease","volume":"56","author":"Wang","year":"2024","journal-title":"Int. Urol. Nephrol."},{"key":"ref_151","doi-asserted-by":"crossref","first-page":"G853","DOI":"10.1152\/ajpgi.00326.2002","article-title":"Novel MUC1 splice variants contribute to mucin overexpression in CFTR-deficient mice","volume":"284","author":"Johansson","year":"2003","journal-title":"Am. J. Physiol. Gastrointest. Liver Physiol."},{"key":"ref_152","doi-asserted-by":"crossref","unstructured":"Wang, Y., Tang, Q., and Sun, Q. (2025). INHBA knockdown inhibits renal fibrosis in mice following ischemia-reperfusion injury by suppressing activation of the TGF-\u03b2\/Smad signaling pathway. BMC Nephrol., 26.","DOI":"10.1186\/s12882-025-04443-2"},{"key":"ref_153","doi-asserted-by":"crossref","unstructured":"Khalaf, M., Scott-Ward, T., Causer, A., Saynor, Z., Shepherd, A., G\u00f3recki, D., Lewis, A., Laight, D., and Shute, J. (2020). Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Human Lung Microvascular Endothelial Cells Controls Oxidative Stress, Reactive Oxygen-Mediated Cell Signaling and Inflammatory Responses. Front. Physiol., 11.","DOI":"10.3389\/fphys.2020.00879"},{"key":"ref_154","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1038\/ncpneuro0883","article-title":"Roles of the cation-chloride cotransporters in neurological disease","volume":"4","author":"Kahle","year":"2008","journal-title":"Nat. Clin. Pract. Neurol."},{"key":"ref_155","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1165\/ajrcmb\/8.2.201","article-title":"Cystic fibrosis gene and protein expression during fetal lung development","volume":"8","author":"McGrath","year":"1993","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_156","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1172\/JCI118080","article-title":"Expression and localization of the cystic fibrosis transmembrane conductance regulator mRNA and its protein in rat brain","volume":"96","author":"Mulberg","year":"1995","journal-title":"J. Clin. Investig."},{"key":"ref_157","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1097\/00001756-199801050-00028","article-title":"Cystic fibrosis transmembrane conductance regulator expression in human hypothalamus","volume":"9","author":"Mulberg","year":"1998","journal-title":"Neuroreport"},{"key":"ref_158","doi-asserted-by":"crossref","first-page":"3083","DOI":"10.1073\/pnas.1222729110","article-title":"CFTR-deficient pigs display peripheral nervous system defects at birth","volume":"110","author":"Reznikov","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_159","doi-asserted-by":"crossref","first-page":"2628","DOI":"10.1038\/ncomms3628","article-title":"Activity-dependent regulation of dendritic growth and maintenance by glycogen synthase kinase 3\u03b2","volume":"4","author":"Rui","year":"2013","journal-title":"Nat. Commun."},{"key":"ref_160","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1038\/nm.2101","article-title":"Pharmacological correction of a defect in PPAR-gamma signaling ameliorates disease severity in Cftr-deficient mice","volume":"16","author":"Harmon","year":"2010","journal-title":"Nat. Med."},{"key":"ref_161","doi-asserted-by":"crossref","first-page":"104384","DOI":"10.1016\/j.ebiom.2022.104384","article-title":"Restoring myocardial infarction-induced long-term memory impairment by targeting the cystic fibrosis transmembrane regulator","volume":"86","author":"Vanherle","year":"2022","journal-title":"eBioMedicine"},{"key":"ref_162","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1161\/STROKEAHA.121.036950","article-title":"Circadian Rhythmicity in Cerebral Microvascular Tone Influences Subarachnoid Hemorrhage-Induced Injury","volume":"53","author":"Lidington","year":"2022","journal-title":"Stroke"},{"key":"ref_163","doi-asserted-by":"crossref","unstructured":"Vij, N., Mazur, S., and Zeitlin, P.L. (2009). CFTR is a negative regulator of NF\u03baB mediated innate immune response. PLoS ONE, 4.","DOI":"10.1371\/journal.pone.0004664"},{"key":"ref_164","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1186\/s12974-021-02325-6","article-title":"Dysregulated phosphoinositide 3-kinase signaling in microglia: Shaping chronic neuroinflammation","volume":"18","author":"Chu","year":"2021","journal-title":"J. Neuroinflamm."},{"key":"ref_165","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1186\/s12974-024-03024-8","article-title":"Modeling the neuroimmune system in Alzheimer\u2019s and Parkinson\u2019s diseases","volume":"21","author":"Balestri","year":"2024","journal-title":"J. Neuroinflamm."},{"key":"ref_166","doi-asserted-by":"crossref","first-page":"3868","DOI":"10.1038\/sj.emboj.7601805","article-title":"Structural and functional insights into RAGE activation by multimeric S100B","volume":"26","author":"Ostendorp","year":"2007","journal-title":"EMBO J."},{"key":"ref_167","doi-asserted-by":"crossref","first-page":"539581","DOI":"10.1155\/2010\/539581","article-title":"The S100B\/RAGE Axis in Alzheimer\u2019s Disease","volume":"2010","author":"Leclerc","year":"2010","journal-title":"Cardiovasc. Psychiatry Neurol."},{"key":"ref_168","doi-asserted-by":"crossref","unstructured":"Valdivieso, A.G., Clauzure, M., Mar\u00edn, M.C., Taminelli, G.L., Massip Copiz, M.M., S\u00e1nchez, F., Schulman, G., Teiber, M.L., and Santa-Coloma, T.A. (2012). The mitochondrial complex I activity is reduced in cells with impaired cystic fibrosis transmembrane conductance regulator (CFTR) function. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0048059"},{"key":"ref_169","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1186\/1750-1326-3-9","article-title":"The role of Wnt signaling in neuronal dysfunction in Alzheimer\u2019s Disease","volume":"3","author":"Inestrosa","year":"2008","journal-title":"Mol. Neurodegener."},{"key":"ref_170","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1165\/rcmb.2014-0263OC","article-title":"Oxidative stress regulates CFTR gene expression in human airway epithelial cells through a distal antioxidant response element","volume":"52","author":"Zhang","year":"2015","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_171","doi-asserted-by":"crossref","unstructured":"Numakawa, T., and Kajihara, R. (2026). Roles of MAPKs, Including Those Activated by BDNF\/TrkB, and Their Contribution in Neurodegenerative Diseases. Int. J. Mol. Sci., 27.","DOI":"10.3390\/ijms27020984"},{"key":"ref_172","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/j.brainres.2008.05.067","article-title":"Cytokine mRNA induction by interleukin-1beta or tumor necrosis factor alpha in vitro and in vivo","volume":"1226","author":"Taishi","year":"2008","journal-title":"Brain Res."},{"key":"ref_173","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1016\/j.neurobiolaging.2008.05.017","article-title":"S100B\/RAGE-dependent activation of microglia via NF-kappaB and AP-1 Co-regulation of COX-2 expression by S100B, IL-1beta and TNF-alpha","volume":"31","author":"Bianchi","year":"2010","journal-title":"Neurobiol. Aging"},{"key":"ref_174","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1016\/j.redox.2012.11.007","article-title":"CFTR activity and mitochondrial function","volume":"1","author":"Valdivieso","year":"2013","journal-title":"Redox Biol."},{"key":"ref_175","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1016\/j.bbrc.2007.11.076","article-title":"CISD1 codifies a mitochondrial protein upregulated by the CFTR channel","volume":"365","author":"Taminelli","year":"2008","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_176","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1038\/sj.jcbfm.9600106","article-title":"NFkappaB activates in vivo the synthesis of inducible Cox-2 in the brain","volume":"25","author":"Nadjar","year":"2005","journal-title":"J. Cereb. Blood Flow Metab."},{"key":"ref_177","doi-asserted-by":"crossref","first-page":"64030","DOI":"10.18632\/oncotarget.11747","article-title":"Defective CFTR- \u03b2-catenin interaction promotes NF-\u03baB nuclear translocation and intestinal inflammation in cystic fibrosis","volume":"7","author":"Liu","year":"2016","journal-title":"Oncotarget"},{"key":"ref_178","doi-asserted-by":"crossref","unstructured":"Lopes-Pacheco, M. (2016). CFTR Modulators: Shedding Light on Precision Medicine for Cystic Fibrosis. Front. Pharmacol., 7.","DOI":"10.3389\/fphar.2016.00275"},{"key":"ref_179","doi-asserted-by":"crossref","unstructured":"Lopes-Pacheco, M. (2019). CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine. Front. Pharmacol., 10.","DOI":"10.3389\/fphar.2019.01662"},{"key":"ref_180","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1056\/NEJMoa1105185","article-title":"A CFTR potentiator in patients with cystic fibrosis and the G551D mutation","volume":"365","author":"Ramsey","year":"2011","journal-title":"N. Engl. J. Med."},{"key":"ref_181","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1056\/NEJMoa1409547","article-title":"Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR","volume":"373","author":"Wainwright","year":"2015","journal-title":"N. Engl. J. Med."},{"key":"ref_182","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1056\/NEJMoa1908639","article-title":"Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele","volume":"381","author":"Middleton","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_183","doi-asserted-by":"crossref","first-page":"1940","DOI":"10.1016\/S0140-6736(19)32597-8","article-title":"Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: A double-blind, randomised, phase 3 trial","volume":"394","author":"Heijerman","year":"2019","journal-title":"Lancet"},{"key":"ref_184","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1056\/NEJMoa2100665","article-title":"Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes","volume":"385","author":"Barry","year":"2021","journal-title":"N. Engl. J. Med."},{"key":"ref_185","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1136\/ebmed-2015-110325","article-title":"Lumacaftor-ivacaftor (Orkambi) for cystic fibrosis: Behind the \u2018breakthrough\u2019","volume":"21","author":"Mayer","year":"2016","journal-title":"BMJ Evid.-Based Med."},{"key":"ref_186","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1513\/AnnalsATS.202002-144OC","article-title":"Clinical effectiveness of lumacaftor\/ivacaftor in patients with cystic fibrosis homozygous for F508del-CFTR. A clinical trial","volume":"18","author":"Sagel","year":"2021","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_187","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1164\/rccm.201704-0717OC","article-title":"Tezacaftor\/ivacaftor in subjects with cystic fibrosis and F508del\/F508del-CFTR or F508del\/G551D-CFTR","volume":"197","author":"Donaldson","year":"2018","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_188","doi-asserted-by":"crossref","first-page":"186","DOI":"10.2967\/jnmt.113.122952","article-title":"Variability of total lesion glycolysis by 18F-FDG-positive tissue thresholding in lung cancer","volume":"41","author":"Laffon","year":"2013","journal-title":"J. Nucl. Med. Technol."},{"key":"ref_189","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1016\/j.stem.2013.11.002","article-title":"Functional repair of CFTR by CRISPR\/Cas9 in intestinal stem cell organoids of cystic fibrosis patients","volume":"13","author":"Schwank","year":"2013","journal-title":"Cell Stem Cell"},{"key":"ref_190","doi-asserted-by":"crossref","unstructured":"Zhou, A., Xiong, C., Hu, R., Zhang, Y., Bassig, B.A., Triche, E., Yang, S., Qiu, L., Zhang, Y., and Yao, C. (2015). Pre-Pregnancy BMI, Gestational Weight Gain, and the Risk of Hypertensive Disorders of Pregnancy: A Cohort Study in Wuhan, China. PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0136291"},{"key":"ref_191","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1164\/rccm.2110015","article-title":"Effects of recombinant human DNase and hypertonic saline on airway inflammation in children with cystic fibrosis","volume":"166","author":"Suri","year":"2002","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_192","doi-asserted-by":"crossref","first-page":"680","DOI":"10.1164\/rccm.201207-1160OE","article-title":"Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health","volume":"187","author":"Mogayzel","year":"2013","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_193","doi-asserted-by":"crossref","first-page":"1677","DOI":"10.1128\/EC.00050-09","article-title":"Reconstruction of signaling networks regulating fungal morphogenesis by transcriptomics","volume":"8","author":"Meyer","year":"2009","journal-title":"Eukaryot. Cell"},{"key":"ref_194","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1016\/j.jada.2008.02.020","article-title":"Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: Results of a systematic review","volume":"108","author":"Stallings","year":"2008","journal-title":"J. Am. Diet. Assoc."},{"key":"ref_195","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1097\/CEJ.0000000000000185","article-title":"The variation trends of SFRP2 methylation of tissue, feces, and blood detection in colorectal cancer development","volume":"25","author":"Sui","year":"2016","journal-title":"Eur. J. Cancer Prev."},{"key":"ref_196","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1016\/j.tips.2007.05.004","article-title":"Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis","volume":"28","author":"Amaral","year":"2007","journal-title":"Trends Pharmacol. Sci."},{"key":"ref_197","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1186\/s13073-015-0223-6","article-title":"Targeted therapies to improve CFTR function in cystic fibrosis","volume":"7","author":"Brodlie","year":"2015","journal-title":"Genome Med."},{"key":"ref_198","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1186\/s12967-017-1193-9","article-title":"Cystic fibrosis: Current therapeutic targets and future approaches","volume":"15","author":"Rafeeq","year":"2017","journal-title":"J. Transl. Med."},{"key":"ref_199","doi-asserted-by":"crossref","first-page":"683","DOI":"10.2174\/138945011795378586","article-title":"Targeting CFTR: How to treat cystic fibrosis by CFTR-repairing therapies","volume":"12","author":"Amaral","year":"2011","journal-title":"Curr. Drug Targets"},{"key":"ref_200","doi-asserted-by":"crossref","first-page":"S14","DOI":"10.1016\/j.jcf.2017.08.013","article-title":"Therapeutic approaches to CFTR dysfunction: From discovery to drug development","volume":"17","author":"Li","year":"2018","journal-title":"J. Cyst. Fibros."},{"key":"ref_201","doi-asserted-by":"crossref","first-page":"686","DOI":"10.4274\/tjps.galenos.2020.76401","article-title":"New therapeutic approaches in cystic fibrosis","volume":"17","author":"Altun","year":"2020","journal-title":"Turk. J. Pharm. Sci."},{"key":"ref_202","doi-asserted-by":"crossref","first-page":"i859","DOI":"10.1136\/bmj.i859","article-title":"New and emerging targeted therapies for cystic fibrosis","volume":"352","author":"Quon","year":"2016","journal-title":"BMJ"},{"key":"ref_203","doi-asserted-by":"crossref","first-page":"690","DOI":"10.4081\/mrm.2020.690","article-title":"Novel therapeutic approaches for the management of cystic fibrosis","volume":"15","author":"Jaques","year":"2020","journal-title":"Multidiscip. Respir. Med."},{"key":"ref_204","doi-asserted-by":"crossref","first-page":"1185","DOI":"10.1016\/S0140-6736(23)01608-2","article-title":"The future of cystic fibrosis treatment: From disease mechanisms to novel therapeutic approaches","volume":"402","author":"Graeber","year":"2023","journal-title":"Lancet"},{"key":"ref_205","doi-asserted-by":"crossref","unstructured":"Ajilore, P.O., Yang, H.Y., Kerasidis, A., and Castro, R. (2022). Curative Measures for Cystic Fibrosis: A Perspective on Current Stem Cell\u2013Based, Gene, and Small Molecule Therapies. Georget. Med. Rev., 6.","DOI":"10.52504\/001c.38728"},{"key":"ref_206","doi-asserted-by":"crossref","first-page":"113195","DOI":"10.1016\/j.ejmech.2021.113195","article-title":"Modulators of CFTR. Updates on clinical development and future directions","volume":"213","author":"Bardin","year":"2021","journal-title":"Eur. J. Med. Chem."},{"key":"ref_207","doi-asserted-by":"crossref","first-page":"7234","DOI":"10.1038\/s41598-019-43652-2","article-title":"A systematic review of the clinical efficacy and safety of CFTR modulators in cystic fibrosis","volume":"9","author":"Habib","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_208","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1136\/thoraxjnl-2015-208123","article-title":"Advancing clinical development pathways for new CFTR modulators in cystic fibrosis","volume":"71","author":"Boyle","year":"2016","journal-title":"Thorax"},{"key":"ref_209","first-page":"939","article-title":"Novel opportunities for CFTR-targeting drug development using organoids","volume":"1","author":"Dekkers","year":"2013","journal-title":"Rare Dis."},{"key":"ref_210","doi-asserted-by":"crossref","first-page":"329","DOI":"10.4155\/fmc.12.1","article-title":"Recent advances and new perspectives in targeting CFTR for therapy of cystic fibrosis and enterotoxin-induced secretory diarrheas","volume":"4","author":"Zhang","year":"2012","journal-title":"Future Med. Chem."},{"key":"ref_211","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1038\/aps.2011.71","article-title":"Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis","volume":"32","author":"Cai","year":"2011","journal-title":"Acta Pharmacol. Sin."},{"key":"ref_212","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1080\/17460441.2021.1912732","article-title":"Discovery of CFTR modulators for the treatment of cystic fibrosis","volume":"16","author":"Pedemonte","year":"2021","journal-title":"Expert Opin. Drug Discov."},{"key":"ref_213","doi-asserted-by":"crossref","first-page":"1035","DOI":"10.1113\/EP085779","article-title":"Temporal partitioning of cardiac metabolism by the cardiomyocyte circadian clock","volume":"101","author":"Young","year":"2016","journal-title":"Exp. Physiol."},{"key":"ref_214","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1016\/j.jcf.2017.09.012","article-title":"Lumacaftor\/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR","volume":"17","author":"Jain","year":"2018","journal-title":"J. Cyst. Fibros."},{"key":"ref_215","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1002\/bcp.70278","article-title":"Pharmacogenomics in clinical practice: Biomarker information in Brazilian drug labels","volume":"92","year":"2026","journal-title":"Br. J. Clin. Pharmacol."},{"key":"ref_216","doi-asserted-by":"crossref","first-page":"1599","DOI":"10.1056\/NEJMoa1807119","article-title":"VX-659-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles","volume":"379","author":"Davies","year":"2018","journal-title":"N. Engl. J. Med."},{"key":"ref_217","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1007\/s40272-022-00509-y","article-title":"An update on CFTR modulators as new therapies for cystic fibrosis","volume":"24","author":"King","year":"2022","journal-title":"Pediatr. Drugs"},{"key":"ref_218","doi-asserted-by":"crossref","unstructured":"Wang, Y., Ma, B., Li, W., and Li, P. (2022). Efficacy and Safety of Triple Combination Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Patients With Cystic Fibrosis: A Meta-Analysis of Randomized Controlled Trials. Front. Pharmacol., 13.","DOI":"10.3389\/fphar.2022.863280"},{"key":"ref_219","doi-asserted-by":"crossref","first-page":"1398","DOI":"10.1164\/rccm.202402-0389CI","article-title":"Lentiviral gene therapy for cystic fibrosis: A promising approach and first-in-human trial","volume":"210","author":"Davies","year":"2024","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_220","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1016\/j.jcf.2018.02.007","article-title":"Comparison of ex vivo and in vitro intestinal cystic fibrosis models to measure CFTR-dependent ion channel activity","volume":"17","author":"Kruisselbrink","year":"2018","journal-title":"J. Cyst. Fibros."},{"key":"ref_221","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1016\/j.jcf.2023.04.003","article-title":"Targeted locus amplification reveals heterogeneity between and within CFTR genotypes and association with CFTR function in patient-derived intestinal organoids","volume":"22","author":"Lefferts","year":"2023","journal-title":"J. Cyst. 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