{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T21:35:09Z","timestamp":1791668109933,"version":"4.3.4"},"reference-count":110,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,11,16]],"date-time":"2023-11-16T00:00:00Z","timestamp":1700092800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Cystic fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the CF Transmembrane-conductance Regulator (CFTR) gene. The most severe pathologies of CF occur in the lung, manifesting as chronic bacterial infection, persistent neutrophilic inflammation, and mucopurulent airway obstruction. Despite increasing knowledge of the CF primary defect and the resulting clinical sequelae, the relationship between the CFTR loss of function and the neutrophilic inflammation remains incompletely understood. Here, we report that loss of CFTR function in macrophages causes extended lung inflammation. After intratracheal inoculation with <jats:italic>Pseudomonas aeruginosa<\/jats:italic>, mice with a macrophage-specific <jats:italic>Cftr<\/jats:italic>-knockout (Mac-CF) were able to mount an effective host defense to clear the bacterial infection. However, three days post-inoculation, Mac-CF lungs demonstrated significantly more neutrophil infiltration and higher levels of inflammatory cytokines, suggesting that Mac-CF mice had a slower resolution of inflammation. Single-cell RNA sequencing revealed that absence of CFTR in the macrophages altered the cell transcriptional program, affecting the cell inflammatory and immune responses, antioxidant system, and mitochondrial respiration. Thus, loss of CFTR function in macrophages influences cell homeostasis, leading to a dysregulated cellular response to infection that may exacerbate CF lung disease.<\/jats:p>","DOI":"10.3389\/fimmu.2023.1242381","type":"journal-article","created":{"date-parts":[[2023,11,16]],"date-time":"2023-11-16T13:52:31Z","timestamp":1700142751000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":13,"title":["Loss of CFTR function in macrophages alters the cell transcriptional program and delays lung resolution of inflammation"],"prefix":"10.3389","volume":"14","author":[{"given":"Dianne","family":"Wellems","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yawen","family":"Hu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott","family":"Jennings","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guoshun","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2023,11,16]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201702-0266ED","article-title":"AJRCCM: 100-year anniversary. Progress along the pathway of discovery leading to treatment and cure of cystic fibrosis","volume":"195","author":"Ramsey","year":"2017","journal-title":"Am J Respir Crit Care Med"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1126\/science.2772657","article-title":"Identification of the cystic fibrosis gene: chromosome walking and jumping","volume":"245","author":"Rommens","year":"1989","journal-title":"Science"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.154.5.8912731","article-title":"Cystic fibrosis","volume":"154","author":"Davis","year":"1996","journal-title":"Am J Respir Crit Care Med"},{"key":"B4","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1016\/j.cell.2017.02.024","article-title":"Molecular structure of the human CFTR ion channel","volume":"169","author":"Liu","year":"2017","journal-title":"Cell"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(20)32542-3","article-title":"Cystic fibrosis","volume":"397","author":"Shteinberg","year":"2021","journal-title":"Lancet"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200505-840OE","article-title":"Cystic fibrosis since 1938","volume":"173","author":"Davis","year":"2006","journal-title":"Am J Resp Crit Care"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24129","article-title":"Inflammation in cystic fibrosis: An update","volume":"53","author":"Roesch","year":"2018","journal-title":"Pediatr Pulmonol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"194","DOI":"10.1128\/CMR.15.2.194-222.2002","article-title":"Lung infections associated with cystic fibrosis","volume":"15","author":"Lyczak","year":"2002","journal-title":"Clin Microbiol Rev"},{"key":"B9","doi-asserted-by":"publisher","first-page":"299","DOI":"10.1128\/CMR.00068-09","article-title":"The changing microbial epidemiology in cystic fibrosis","volume":"23","author":"Lipuma","year":"2010","journal-title":"Clin Microbiol Rev"},{"key":"B10","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1016\/j.ccm.2015.10.003","article-title":"The microbiome in cystic fibrosis","volume":"37","author":"Huang","year":"2016","journal-title":"Clin Chest Med"},{"key":"B11","unstructured":"RegistryCFP\n          Patient registry annual data report 20192019"},{"key":"B12","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1002\/ppul.10127","article-title":"Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis","volume":"34","author":"Emerson","year":"2002","journal-title":"Pediatr Pulmonol"},{"key":"B13","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1016\/j.jcf.2016.07.012","article-title":"Reduced survival in adult cystic fibrosis despite attenuated lung function decline","volume":"16","author":"Keating","year":"2017","journal-title":"J Cyst Fibros"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.2009.abs","article-title":"Acceleration of lung disease in children with cystic fibrosis after Pseudomonas aeruginosa acquisition","volume":"32","author":"Kosorok","year":"2001","journal-title":"Pediatr Pulmonol"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1813799","article-title":"Muco-obstructive lung diseases","volume":"380","author":"Boucher","year":"2019","journal-title":"N Engl J Med"},{"key":"B16","doi-asserted-by":"publisher","first-page":"792","DOI":"10.1016\/j.immuni.2013.04.004","article-title":"Tissue-resident macrophages self-maintain locally throughout adult life with minimal contribution from circulating monocytes","volume":"38","author":"Hashimoto","year":"2013","journal-title":"Immunity"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2015-207020","article-title":"Pulmonary macrophages: key players in the innate defence of the airways","volume":"70","author":"Byrne","year":"2015","journal-title":"Thorax"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.0907658","article-title":"The role of chloride anion and CFTR in killing of Pseudomonas aeruginosa by normal and CF neutrophils","volume":"83","author":"Painter","year":"2008","journal-title":"J Leukoc Biol"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1021\/bi060490t","article-title":"CFTR Expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis","volume":"45","author":"Painter","year":"2006","journal-title":"Biochemistry"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1016\/j.freeradbiomed.2020.03.001","article-title":"Myeloperoxidase inhibition decreases morbidity and oxidative stress in mice with cystic fibrosis-like lung inflammation","volume":"152","author":"Dickerhof","year":"2020","journal-title":"Free Radic Biol Med"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.3389\/fcimb.2017.00243","article-title":"Harnessing Neutrophil Survival Mechanisms during Chronic Infection by Pseudomonas aeruginosa: Novel Therapeutic Targets to Dampen Inflammation in Cystic Fibrosis","volume":"7","author":"Marteyn","year":"2017","journal-title":"Front Cell Infect Microbiol"},{"key":"B22","doi-asserted-by":"publisher","first-page":"10935","DOI":"10.1038\/s41598-020-67729-5","article-title":"Altered iron metabolism in cystic fibrosis macrophages: the impact of CFTR modulators and implications for Pseudomonas aeruginosa survival","volume":"10","author":"Hazlett","year":"2020","journal-title":"Sci Rep"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.01789","article-title":"Metabolic reprograming of cystic fibrosis macrophages via the IRE1alpha arm of the unfolded protein response results in exacerbated inflammation","volume":"10","author":"Lara-Reyna","year":"2019","journal-title":"Front Immunol"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0075667","article-title":"Impaired functions of macrophage from cystic fibrosis patients: CD11b, TLR-5 decrease and sCD14, inflammatory cytokines increase","volume":"8","author":"Simonin-Le Jeune","year":"2013","journal-title":"PloS One"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.00030.2009","article-title":"Dysfunctional cystic fibrosis transmembrane conductance regulator inhibits phagocytosis of apoptotic cells with proinflammatory consequences","volume":"297","author":"Vandivier","year":"2009","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1172\/jci.insight.152186","article-title":"CFTR-mediated monocyte\/macrophage dysfunction revealed by cystic fibrosis proband-parent comparisons","volume":"7","author":"Zhang","year":"2022","journal-title":"JCI Insight"},{"key":"B27","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1165\/rcmb.2008-0170OC","article-title":"Macrophages directly contribute to the exaggerated inflammatory response in cystic fibrosis transmembrane conductance regulator-\/- mice","volume":"40","author":"Bruscia","year":"2009","journal-title":"Am J Respir Cell Mol Biol"},{"key":"B28","doi-asserted-by":"publisher","first-page":"111797","DOI":"10.1016\/j.celrep.2022.111797","article-title":"Recruited monocytes\/macrophages drive pulmonary neutrophilic inflammation and irreversible lung tissue remodeling in cystic fibrosis","volume":"41","author":"Oz","year":"2022","journal-title":"Cell Rep"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1189\/jlb.0412188","article-title":"Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection","volume":"92","author":"Bonfield","year":"2012","journal-title":"J Leukoc Biol"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0106813","article-title":"Neutrophil-mediated phagocytic host defense defect in myeloid cftr-inactivated mice","volume":"9","author":"Ng","year":"2014","journal-title":"PloS One"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1002\/JLB.3A0520-193RR","article-title":"Myeloid CFTR loss-of-function causes persistent neutrophilic inflammation in cystic fibrosis","volume":"108","author":"Ng","year":"2020","journal-title":"J Leukoc Biol"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1002\/dvg.20433","article-title":"Generation of a conditional null allele for Cftr in mice","volume":"46","author":"Hodges","year":"2008","journal-title":"Genesis"},{"key":"B33","doi-asserted-by":"publisher","first-page":"79","DOI":"10.1016\/j.immuni.2012.12.001","article-title":"Fate mapping reveals origins and dynamics of monocytes and tissue macrophages under homeostasis","volume":"38","author":"Yona","year":"2013","journal-title":"Immunity"},{"key":"B34","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1007\/978-1-4939-7837-3_3","article-title":"Growing murine bone marrow-derived macrophages","volume":"1784","author":"Assouvie","year":"2018","journal-title":"Macrophages: Springer"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0015263","article-title":"A method for generation of bone marrow-derived macrophages from cryopreserved mouse bone marrow cells","volume":"5","author":"Marim","year":"2010","journal-title":"PloS One"},{"key":"B36","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1038\/s41420-020-00300-3","article-title":"Establishment of bone marrow-derived M-CSF receptor-dependent self-renewing macrophages","volume":"6","author":"Nasser","year":"2020","journal-title":"Cell Death Discovery"},{"key":"B37","doi-asserted-by":"publisher","first-page":"pdb prot5080","DOI":"10.1101\/pdb.prot5080","article-title":"Bone marrow-derived macrophages (BMM): isolation and applications","volume":"2008","author":"Weischenfeldt","year":"2008","journal-title":"CSH Protoc"},{"key":"B38","volume-title":"R: A language and environment for statistical computing","year":"2013"},{"key":"B39","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2021.04.048","article-title":"Integrated analysis of multimodal single-cell data","volume":"184","author":"Hao","year":"2021","journal-title":"Cell"},{"key":"B40","doi-asserted-by":"publisher","first-page":"1888","DOI":"10.1016\/j.cell.2019.05.031","article-title":"Comprehensive integration of single-cell data","volume":"177","author":"Stuart","year":"2019","journal-title":"Cell"},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1038\/nbt.4096","article-title":"Integrating single-cell transcriptomic data across different conditions, technologies, and species","volume":"36","author":"Butler","year":"2018","journal-title":"Nat Biotechnol"},{"key":"B42","doi-asserted-by":"publisher","first-page":"495","DOI":"10.1038\/nbt.3192","article-title":"Spatial reconstruction of single-cell gene expression data","volume":"33","author":"Satija","year":"2015","journal-title":"Nat Biotechnol"},{"key":"B43","volume-title":"RStudio: integrated development for R","year":"2020"},{"key":"B44","doi-asserted-by":"publisher","first-page":"296","DOI":"10.1186\/s13059-019-1874-1","article-title":"Normalization and variance stabilization of single-cell RNA-seq data using regularized negative binomial regression","volume":"20","author":"Hafemeister","year":"2019","journal-title":"Genome Biol"},{"key":"B45","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1186\/s13059-021-02584-9","article-title":"Comparison and evaluation of statistical error models for scRNA-seq","volume":"23","author":"Choudhary","year":"2022","journal-title":"Genome Biol"},{"key":"B46","doi-asserted-by":"publisher","DOI":"10.1038\/s41592-019-0619-0","article-title":"Fast, sensitive and accurate integration of single-cell data with Harmony","volume":"16","author":"Korsunsky","year":"2019","journal-title":"Nat Methods"},{"key":"B47","doi-asserted-by":"publisher","DOI":"10.1038\/ni1008-1091","article-title":"The Immunological Genome Project: networks of gene expression in immune cells","volume":"9","author":"Heng","year":"2008","journal-title":"Nat Immunol"},{"key":"B48","doi-asserted-by":"publisher","DOI":"10.1038\/s41590-018-0276-y","article-title":"Reference-based analysis of lung single-cell sequencing reveals a transitional profibrotic macrophage","volume":"20","author":"Aran","year":"2019","journal-title":"Nat Immunol"},{"key":"B49","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1502364","article-title":"Expression profiling of macrophages reveals multiple populations with distinct biological roles in an immunocompetent orthotopic model of lung cancer","volume":"196","author":"Poczobutt","year":"2016","journal-title":"J Immunol"},{"key":"B50","doi-asserted-by":"publisher","DOI":"10.1038\/s41590-019-0568-x","article-title":"Influenza-induced monocyte-derived alveolar macrophages confer prolonged antibacterial protection","volume":"21","author":"Aegerter","year":"2020","journal-title":"Nat Immunol"},{"key":"B51","doi-asserted-by":"publisher","first-page":"14348","DOI":"10.1038\/s41598-020-71144-1","article-title":"Differential contribution of bone marrow-derived infiltrating monocytes and resident macrophages to persistent lung inflammation in chronic air pollution exposure","volume":"10","author":"Gangwar","year":"2020","journal-title":"Sci Rep"},{"key":"B52","doi-asserted-by":"publisher","DOI":"10.3791\/55398","article-title":"Bronchoalveolar lavage of murine lungs to analyze inflammatory cell infiltration","volume":"123","author":"Van Hoecke","year":"2017","journal-title":"J Vis Exp"},{"key":"B53","doi-asserted-by":"publisher","first-page":"100141","DOI":"10.1016\/j.xinn.2021.100141","article-title":"clusterProfiler 4.0: A universal enrichment tool for interpreting omics data","volume":"2","author":"Wu","year":"2021","journal-title":"Innovation (Camb)"},{"key":"B54","doi-asserted-by":"publisher","DOI":"10.1089\/omi.2011.0118","article-title":"clusterProfiler: an R package for comparing biological themes among gene clusters","volume":"16","author":"Yu","year":"2012","journal-title":"Omics"},{"key":"B55","volume-title":"org. Mm. eg. db: Genome wide annotation for Mouse. R package version 3.8.2","author":"Carlson","year":"2019"},{"key":"B56","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0506580102","article-title":"Gene set enrichment analysis: a knowledge-based approach for interpreting genome-wide expression profiles","volume":"102","author":"Subramanian","year":"2005","journal-title":"Proc Natl Acad Sci USA"},{"key":"B57","doi-asserted-by":"publisher","DOI":"10.1016\/j.cels.2015.12.004","article-title":"The Molecular Signatures Database (MSigDB) hallmark gene set collection","volume":"1","author":"Liberzon","year":"2015","journal-title":"Cell Syst"},{"key":"B58","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btr260","article-title":"Molecular signatures database (MSigDB) 3","volume":"27","author":"Liberzon","year":"2011","journal-title":"0. Bioinf"},{"key":"B59","doi-asserted-by":"publisher","DOI":"10.1038\/ng1180","article-title":"PGC-1alpha-responsive genes involved in oxidative phosphorylation are coordinately downregulated in human diabetes","volume":"34","author":"Mootha","year":"2003","journal-title":"Nat Genet"},{"key":"B60","doi-asserted-by":"publisher","DOI":"10.1038\/nmeth.4463","article-title":"SCENIC: single-cell regulatory network inference and clustering","volume":"14","author":"Aibar","year":"2017","journal-title":"Nat Methods"},{"key":"B61","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.56890.sa2","article-title":"Diverse homeostatic and immunomodulatory roles of immune cells in the developing mouse lung at single cell resolution","volume":"9","author":"Domingo-Gonzalez","year":"2020","journal-title":"Elife"},{"key":"B62","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/2050-7771-2-1","article-title":"Monocyte and macrophage differentiation: circulation inflammatory monocyte as biomarker for inflammatory diseases","volume":"2","author":"Yang","year":"2014","journal-title":"biomark Res"},{"key":"B63","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2021.620510","article-title":"Function of macrophages in disease: current understanding on molecular mechanisms","volume":"12","author":"Zhang","year":"2021","journal-title":"Front Immunol"},{"key":"B64","doi-asserted-by":"publisher","DOI":"10.1172\/jci.insight.126556","article-title":"Single cell RNA sequencing identifies unique inflammatory airspace macrophage subsets","volume":"4","author":"Mould","year":"2019","journal-title":"JCI Insight"},{"key":"B65","doi-asserted-by":"publisher","first-page":"2","DOI":"10.1186\/1465-9921-10-2","article-title":"Transcriptome profiling of primary murine monocytes, lung macrophages and lung dendritic cells reveals a distinct expression of genes involved in cell trafficking","volume":"10","author":"Zaslona","year":"2009","journal-title":"Respir Res"},{"key":"B66","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.117.312509","article-title":"Single-cell RNA-seq reveals the transcriptional landscape and heterogeneity of aortic macrophages in murine atherosclerosis","volume":"122","author":"Cochain","year":"2018","journal-title":"Circ Res"},{"key":"B67","doi-asserted-by":"publisher","DOI":"10.2174\/092986709787846578","article-title":"Mitochondrial respiratory complex I: structure, function and implication in human diseases","volume":"16","author":"Sharma","year":"2009","journal-title":"Curr Med Chem"},{"key":"B68","doi-asserted-by":"publisher","DOI":"10.1007\/s11064-008-9747-0","article-title":"Generation of reactive oxygen species by mitochondrial complex I: implications in neurodegeneration","volume":"33","author":"Fato","year":"2008","journal-title":"Neurochem Res"},{"key":"B69","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1007\/978-3-642-77200-9_5","article-title":"The respiratory-chain NADH dehydrogenase (complex I) of mitochondria","volume":"1992","author":"Weiss","year":"1991","journal-title":"EJB Rev"},{"key":"B70","doi-asserted-by":"publisher","DOI":"10.1016\/j.biocel.2016.06.015","article-title":"Mitochondrial reactive oxygen species and inflammation: Molecular mechanisms, diseases and promising therapies","volume":"81","author":"Rimessi","year":"2016","journal-title":"Int J Biochem Cell Biol"},{"key":"B71","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajme.2017.09.001","article-title":"First line defence antioxidants-superoxide dismutase (SOD), catalase (CAT) and glutathione peroxidase (GPX): Their fundamental role in the entire antioxidant defence grid","volume":"54","author":"Ighodaro","year":"2018","journal-title":"Alexandria J Med"},{"key":"B72","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.mam.2008.08.006","article-title":"Glutathione: overview of its protective roles, measurement, and biosynthesis","volume":"30","author":"Forman","year":"2009","journal-title":"Mol Aspects Med"},{"key":"B73","doi-asserted-by":"publisher","DOI":"10.3389\/fnut.2022.1007816","article-title":"Glutathione: A Samsonian life-sustaining small molecule that protects against oxidative stress, ageing and damaging inflammation","volume":"9","author":"Labarrere","year":"2022","journal-title":"Front Nutr"},{"key":"B74","doi-asserted-by":"publisher","DOI":"10.1038\/ni.1633","article-title":"Superoxide dismutase 1 regulates caspase-1 and endotoxic shock","volume":"9","author":"Meissner","year":"2008","journal-title":"Nat Immunol"},{"key":"B75","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.pharmtox.010909.105600","article-title":"Mechanisms of cell protection by heme oxygenase-1","volume":"50","author":"Gozzelino","year":"2010","journal-title":"Annu Rev Pharmacol Toxicol"},{"key":"B76","doi-asserted-by":"publisher","first-page":"732","DOI":"10.3390\/toxins13100732","article-title":"The protective effect of heme oxygenase-1 on liver injury caused by DON-induced oxidative stress and cytotoxicity","volume":"13","author":"Meng","year":"2021","journal-title":"Toxins (Basel)"},{"key":"B77","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.273.11.6297","article-title":"Mammalian peroxiredoxin isoforms can reduce hydrogen peroxide generated in response to growth factors and tumor necrosis factor-\u03b1","volume":"273","author":"Kang","year":"1998","journal-title":"J Biol Chem"},{"key":"B78","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M407707200","article-title":"Peroxiredoxin III, a mitochondrion-specific peroxidase, regulates apoptotic signaling by mitochondria","volume":"279","author":"Chang","year":"2004","journal-title":"J Biol Chem"},{"key":"B79","doi-asserted-by":"publisher","DOI":"10.1038\/s12276-022-00770-8","article-title":"Recruitment of monocytes primed to express heme oxygenase-1 ameliorates pathological lung inflammation in cystic fibrosis","volume":"54","author":"Di Pietro","year":"2022","journal-title":"Exp Mol Med"},{"key":"B80","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2020.581114","article-title":"Mitochondrial stress responses and \"Mito-inflammation\" in cystic fibrosis","volume":"11","author":"Patergnani","year":"2020","journal-title":"Front Pharmacol"},{"key":"B81","doi-asserted-by":"publisher","first-page":"6201","DOI":"10.1038\/ncomms7201","article-title":"Mitochondrial Ca2+-dependent NLRP3 activation exacerbates the Pseudomonas aeruginosa-driven inflammatory response in cystic fibrosis","volume":"6","author":"Rimessi","year":"2015","journal-title":"Nat Commun"},{"key":"B82","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.aav4634","article-title":"CFTR-PTEN-dependent mitochondrial metabolic dysfunction promotes Pseudomonas aeruginosa airway infection","volume":"11","author":"Riquelme","year":"2019","journal-title":"Sci Transl Med"},{"key":"B83","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202108-1986OC","article-title":"Clinical effectiveness of elexacaftor\/tezacaftor\/ivacaftor in people with cystic fibrosis: A clinical trial","volume":"205","author":"Nichols","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"key":"B84","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202008-3176LE","article-title":"Safety and efficacy of elexacaftor\/tezacaftor\/ivacaftor for 24 weeks or longer in people with cystic fibrosis and one or more F508del alleles: interim results of an open-label phase 3 clinical trial","volume":"203","author":"Griese","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"B85","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201609-1954OC","article-title":"Restoring cystic fibrosis transmembrane conductance regulator function reduces airway bacteria and inflammation in people with cystic fibrosis and chronic lung infections","volume":"195","author":"Hisert","year":"2017","journal-title":"Am J Respir Crit Care Med"},{"key":"B86","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0071717","article-title":"Reactive-oxygen-species-mediated P. aeruginosa killing is functional in human cystic fibrosis macrophages","volume":"8","author":"Cifani","year":"2013","journal-title":"PloS One"},{"key":"B87","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0096681","article-title":"IFN-gamma stimulates autophagy-mediated clearance of Burkholderia cenocepacia in human cystic fibrosis macrophages","volume":"9","author":"Assani","year":"2014","journal-title":"PloS One"},{"key":"B88","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0019970","article-title":"Dysfunctional CFTR alters the bactericidal activity of human macrophages against Pseudomonas aeruginosa","volume":"6","author":"Porto","year":"2011","journal-title":"PloS One"},{"key":"B89","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2015.03.003","article-title":"Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy","volume":"14","author":"Cantin","year":"2015","journal-title":"J Cyst Fibros"},{"key":"B90","doi-asserted-by":"publisher","first-page":"1890","DOI":"10.3390\/jcm8111890","article-title":"An intriguing involvement of mitochondria in cystic fibrosis","volume":"8","author":"Favia","year":"2019","journal-title":"J Clin Med"},{"key":"B91","doi-asserted-by":"publisher","first-page":"740","DOI":"10.3390\/jcm9030740","article-title":"The role of mitochondria in inflammation: from cancer to neurodegenerative disorders","volume":"9","author":"Missiroli","year":"2020","journal-title":"J Clin Med"},{"key":"B92","doi-asserted-by":"publisher","first-page":"132931","DOI":"10.1016\/j.neulet.2017.06.050","article-title":"Inflammation and mitochondrial dysfunction: A vicious circle in neurodegenerative disorders","volume":"710","author":"van Horssen","year":"2019","journal-title":"Neurosci Lett"},{"key":"B93","doi-asserted-by":"publisher","first-page":"21","DOI":"10.3109\/10408449309104073","article-title":"Free radicals as mediators of tissue injury and disease","volume":"23","author":"Kehrer","year":"1993","journal-title":"Crit Rev Toxicology"},{"key":"B94","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/cdg194","article-title":"CFTR directly mediates nucleotide-regulated glutathione flux","volume":"22","author":"Kogan","year":"2003","journal-title":"EMBO J"},{"key":"B95","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1998.275.1.C323","article-title":"Glutathione permeability of CFTR","volume":"275","author":"Linsdell","year":"1998","journal-title":"Am J Physiol"},{"key":"B96","doi-asserted-by":"publisher","DOI":"10.1016\/j.freeradbiomed.2017.09.028","article-title":"Oxidative stress in early cystic fibrosis lung disease is exacerbated by airway glutathione deficiency","volume":"113","author":"Dickerhof","year":"2017","journal-title":"Free Radic Biol Med"},{"key":"B97","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddr173","article-title":"GSH monoethyl ester rescues mitochondrial defects in cystic fibrosis models","volume":"20","author":"Kelly-Aubert","year":"2011","journal-title":"Hum Mol Genet"},{"key":"B98","doi-asserted-by":"publisher","DOI":"10.14814\/phy2.13086","article-title":"Impaired defenses of neonatal mouse alveolar macrophage with cftr deletion are modulated by glutathione and TGFbeta1","volume":"5","author":"Gauthier","year":"2017","journal-title":"Physiol Rep"},{"key":"B99","doi-asserted-by":"publisher","first-page":"3328","DOI":"10.3390\/ijms20133328","article-title":"The NLRP3 inflammasome: an overview of mechanisms of activation and regulation","volume":"20","author":"Kelley","year":"2019","journal-title":"Int J Mol Sci"},{"key":"B100","doi-asserted-by":"publisher","DOI":"10.1038\/s41577-019-0165-0","article-title":"The NLRP3 inflammasome: molecular activation and regulation to therapeutics","volume":"19","author":"Swanson","year":"2019","journal-title":"Nat Rev Immunol"},{"key":"B101","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1038\/nri.2015.7","article-title":"Converging roles of caspases in inflammasome activation, cell death and innate immunity","volume":"16","author":"Man","year":"2016","journal-title":"Nat Rev Immunol"},{"key":"B102","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2012.07.007","article-title":"TRIF licenses caspase-11-dependent NLRP3 inflammasome activation by gram-negative bacteria","volume":"150","author":"Rathinam","year":"2012","journal-title":"Cell"},{"key":"B103","doi-asserted-by":"publisher","first-page":"36","DOI":"10.1038\/s41421-019-0135-5","article-title":"Inflammasome activation and regulation: toward a better understanding of complex mechanisms","volume":"6","author":"Zheng","year":"2020","journal-title":"Cell Discovery"},{"key":"B104","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1128\/IAI.00945-15","article-title":"Pseudomonas aeruginosa triggers macrophage autophagy to escape intracellular killing by activation of the NLRP3 inflammasome","volume":"84","author":"Deng","year":"2016","journal-title":"Infect Immun"},{"key":"B105","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.49248","article-title":"ENaC-mediated sodium influx exacerbates NLRP3-dependent inflammation in cystic fibrosis","volume":"8","author":"Scambler","year":"2019","journal-title":"Elife."},{"key":"B106","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1186\/s13578-019-0292-0","article-title":"Caspase-11, a specific sensor for intracellular lipopolysaccharide recognition, mediates the non-canonical inflammatory pathway of pyroptosis","volume":"9","author":"Huang","year":"2019","journal-title":"Cell Biosci"},{"key":"B107","doi-asserted-by":"publisher","DOI":"10.1038\/nature10558","article-title":"Non-canonical inflammasome activation targets caspase-11","volume":"479","author":"Kayagaki","year":"2011","journal-title":"Nature."},{"key":"B108","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.2000373","article-title":"Function and regulation of noncanonical caspase-4\/5\/11 inflammasome","volume":"204","author":"Matikainen","year":"2020","journal-title":"J Immunol"},{"key":"B109","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.202106-1426OC","article-title":"Trikafta rescues CFTR and lowers monocyte P2X7R-induced inflammasome activation in cystic fibrosis","volume":"205","author":"Gabillard-Lefort","year":"2022","journal-title":"Am J Resp Crit Care"},{"key":"B110","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.54556","article-title":"Different CFTR modulator combinations downregulate inflammation differently in cystic fibrosis","volume":"9","author":"Jarosz-Griffiths","year":"2020","journal-title":"Elife."}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2023.1242381\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,16]],"date-time":"2023-11-16T13:52:41Z","timestamp":1700142761000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2023.1242381\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11,16]]},"references-count":110,"alternative-id":["10.3389\/fimmu.2023.1242381"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2023.1242381","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,11,16]]},"article-number":"1242381"}}