{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T11:48:48Z","timestamp":1790768928903,"version":"4.1.0"},"reference-count":40,"publisher":"MDPI AG","issue":"24","license":[{"start":{"date-parts":[[2022,12,12]],"date-time":"2022-12-12T00:00:00Z","timestamp":1670803200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Science and Technology Department of Sichuan Province","award":["22ZDZX0014"],"award-info":[{"award-number":["22ZDZX0014"]}]},{"name":"Science and Technology Department of Sichuan Province","award":["KL118"],"award-info":[{"award-number":["KL118"]}]},{"name":"Science and Technology Department of Sichuan Province","award":["SCU2022D022"],"award-info":[{"award-number":["SCU2022D022"]}]},{"name":"Science and Technology Department of Sichuan Province","award":["U21A20333"],"award-info":[{"award-number":["U21A20333"]}]},{"name":"Science and Technology Department of Sichuan Province","award":["2021ZYD0105"],"award-info":[{"award-number":["2021ZYD0105"]}]},{"name":"West China Second University Hospital of Sichuan University","award":["22ZDZX0014"],"award-info":[{"award-number":["22ZDZX0014"]}]},{"name":"West China Second University Hospital of Sichuan University","award":["KL118"],"award-info":[{"award-number":["KL118"]}]},{"name":"West China Second University Hospital of Sichuan University","award":["SCU2022D022"],"award-info":[{"award-number":["SCU2022D022"]}]},{"name":"West China Second University Hospital of Sichuan University","award":["U21A20333"],"award-info":[{"award-number":["U21A20333"]}]},{"name":"West China Second University Hospital of Sichuan University","award":["2021ZYD0105"],"award-info":[{"award-number":["2021ZYD0105"]}]},{"name":"Fundamental Research Funds for the Central Universities","award":["22ZDZX0014"],"award-info":[{"award-number":["22ZDZX0014"]}]},{"name":"Fundamental Research Funds for the Central Universities","award":["KL118"],"award-info":[{"award-number":["KL118"]}]},{"name":"Fundamental Research Funds for the Central Universities","award":["SCU2022D022"],"award-info":[{"award-number":["SCU2022D022"]}]},{"name":"Fundamental Research Funds for the Central Universities","award":["U21A20333"],"award-info":[{"award-number":["U21A20333"]}]},{"name":"Fundamental Research Funds for the Central Universities","award":["2021ZYD0105"],"award-info":[{"award-number":["2021ZYD0105"]}]},{"name":"National Natural Science Foundation of China Joint Fund for Regional Innovation and Development","award":["22ZDZX0014"],"award-info":[{"award-number":["22ZDZX0014"]}]},{"name":"National Natural Science Foundation of China Joint Fund for Regional Innovation and Development","award":["KL118"],"award-info":[{"award-number":["KL118"]}]},{"name":"National Natural Science Foundation of China Joint Fund for Regional Innovation and Development","award":["SCU2022D022"],"award-info":[{"award-number":["SCU2022D022"]}]},{"name":"National Natural Science Foundation of China Joint Fund for Regional Innovation and Development","award":["U21A20333"],"award-info":[{"award-number":["U21A20333"]}]},{"name":"National Natural Science Foundation of China Joint Fund for Regional Innovation and Development","award":["2021ZYD0105"],"award-info":[{"award-number":["2021ZYD0105"]}]},{"name":"Central Government Funds of Guiding Local Scientific and Technological Development for Sichuan Province","award":["22ZDZX0014"],"award-info":[{"award-number":["22ZDZX0014"]}]},{"name":"Central Government Funds of Guiding Local Scientific and Technological Development for Sichuan Province","award":["KL118"],"award-info":[{"award-number":["KL118"]}]},{"name":"Central Government Funds of Guiding Local Scientific and Technological Development for Sichuan Province","award":["SCU2022D022"],"award-info":[{"award-number":["SCU2022D022"]}]},{"name":"Central Government Funds of Guiding Local Scientific and Technological Development for Sichuan Province","award":["U21A20333"],"award-info":[{"award-number":["U21A20333"]}]},{"name":"Central Government Funds of Guiding Local Scientific and Technological Development for Sichuan Province","award":["2021ZYD0105"],"award-info":[{"award-number":["2021ZYD0105"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Dynein axonemal heavy chain 5 (DNAH5) is the most mutated gene in primary ciliary dyskinesia (PCD), leading to abnormal cilia ultrastructure and function. Few studies have revealed the genetic characteristics and pathogenetic mechanisms of PCD caused by DNAH5 mutation. Here, we established a child PCD airway organoid directly from the bronchoscopic biopsy of a patient with the DNAH5 mutation. The motile cilia in the organoid were observed and could be stably maintained for an extended time. We further found abnormal ciliary function and a decreased immune response caused by the DNAH5 mutation through single-cell RNA sequencing (scRNA-Seq) and proteomic analyses. Additionally, the directed induction of the ciliated cells, regulated by TGF-\u03b2\/BMP and the Notch pathway, also increased the expression of inflammatory cytokines. Taken together, these results demonstrated that the combination of multiomics analysis and organoid modelling could reveal the close connection between the immune response and the DNAH5 gene.<\/jats:p>","DOI":"10.3390\/cells11244013","type":"journal-article","created":{"date-parts":[[2022,12,12]],"date-time":"2022-12-12T03:36:09Z","timestamp":1670816169000},"page":"4013","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Multiomics Analysis of a DNAH5-Mutated PCD Organoid Model Revealed the Key Role of the TGF-\u03b2\/BMP and Notch Pathways in Epithelial Differentiation and the Immune Response in DNAH5-Mutated Patients"],"prefix":"10.3390","volume":"11","author":[{"given":"Wenhao","family":"Yang","sequence":"first","affiliation":[{"name":"Department of Paediatric Pulmonology and Immunology, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610000, China"},{"name":"NHC Key Laboratory of Chronobiology (Sichuan University), Chengdu 610000, China"},{"name":"The Joint Laboratory for Lung Development and Related Diseases of West China Second University Hospital, Sichuan University and School of Life Sciences of Fudan University, West China Institute of Women and Children\u2019s Health, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Sichuan Birth Defects Clinical Research Centre, West China Second University Hospital, Sichuan University, Chengdu 610000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lina","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Paediatric Pulmonology and Immunology, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610000, China"},{"name":"NHC Key Laboratory of Chronobiology (Sichuan University), Chengdu 610000, China"},{"name":"The Joint Laboratory for Lung Development and Related Diseases of West China Second University Hospital, Sichuan University and School of Life Sciences of Fudan University, West China Institute of Women and Children\u2019s Health, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Sichuan Birth Defects Clinical Research Centre, West China Second University Hospital, Sichuan University, Chengdu 610000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juncen","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Obstetrics\/Gynaecology, Joint Laboratory of Reproductive Medicine (SCU-CUHK), Key Laboratory of Obstetric, Gynaecologic, and Paediatric Diseases and Birth Defects of Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu 610000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fang","family":"Shi","sequence":"additional","affiliation":[{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610000, China"},{"name":"NHC Key Laboratory of Chronobiology (Sichuan University), Chengdu 610000, China"},{"name":"The Joint Laboratory for Lung Development and Related Diseases of West China Second University Hospital, Sichuan University and School of Life Sciences of Fudan University, West China Institute of Women and Children\u2019s Health, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Sichuan Birth Defects Clinical Research Centre, West China Second University Hospital, Sichuan University, Chengdu 610000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qingxin","family":"Yang","sequence":"additional","affiliation":[{"name":"Sichuan Birth Defects Clinical Research Centre, West China Second University Hospital, Sichuan University, Chengdu 610000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liang","family":"Xie","sequence":"additional","affiliation":[{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610000, China"},{"name":"NHC Key Laboratory of Chronobiology (Sichuan University), Chengdu 610000, China"},{"name":"The Joint Laboratory for Lung Development and Related Diseases of West China Second University Hospital, Sichuan University and School of Life Sciences of Fudan University, West China Institute of Women and Children\u2019s Health, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Sichuan Birth Defects Clinical Research Centre, West China Second University Hospital, Sichuan University, Chengdu 610000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danli","family":"Lu","sequence":"additional","affiliation":[{"name":"Department of Paediatric Pulmonology and Immunology, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610000, China"},{"name":"NHC Key Laboratory of Chronobiology (Sichuan University), Chengdu 610000, China"},{"name":"The Joint Laboratory for Lung Development and Related Diseases of West China Second University Hospital, Sichuan University and School of Life Sciences of Fudan University, West China Institute of Women and Children\u2019s Health, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Sichuan Birth Defects Clinical Research Centre, 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School of Life Sciences of Fudan University, West China Institute of Women and Children\u2019s Health, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Sichuan Birth Defects Clinical Research Centre, West China Second University Hospital, Sichuan University, Chengdu 610000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Wang","sequence":"additional","affiliation":[{"name":"The Joint Laboratory for Lung Development and Related Diseases of West China Second University Hospital, Sichuan University and School of Life Sciences of Fudan University, West China Institute of Women and Children\u2019s Health, West China Second University Hospital, Sichuan University, Chengdu 610000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Qiu","sequence":"additional","affiliation":[{"name":"Department of Paediatric Pulmonology and Immunology, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610000, China"},{"name":"NHC Key Laboratory of Chronobiology (Sichuan University), Chengdu 610000, China"},{"name":"The Joint Laboratory for Lung Development and Related Diseases of West China Second University Hospital, Sichuan University and School of Life Sciences of Fudan University, West China Institute of Women and Children\u2019s Health, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Sichuan Birth Defects Clinical Research Centre, West China Second University Hospital, Sichuan University, Chengdu 610000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ting","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Paediatric Pulmonology and Immunology, West China Second University Hospital, Sichuan University, Chengdu 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Chengdu 610000, China"},{"name":"The Joint Laboratory for Lung Development and Related Diseases of West China Second University Hospital, Sichuan University and School of Life Sciences of Fudan University, West China Institute of Women and Children\u2019s Health, West China Second University Hospital, Sichuan University, Chengdu 610000, China"},{"name":"Sichuan Birth Defects Clinical Research Centre, West China Second University Hospital, Sichuan University, Chengdu 610000, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,12,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1248","DOI":"10.1183\/09031936.00001010","article-title":"Factors influencing age at diagnosis of primary ciliary dyskinesia in European children","volume":"36","author":"Kuehni","year":"2010","journal-title":"Eur. Respir. J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1136\/adc.2009.158493","article-title":"High prevalence of primary ciliary dyskinesia in a British Asian population","volume":"95","author":"Chetcuti","year":"2010","journal-title":"Arch. Dis. Child."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"67","DOI":"10.2147\/TACG.S127129","article-title":"Primary ciliary dyskinesia: Mechanisms and management","volume":"10","author":"Damseh","year":"2017","journal-title":"Appl. Clin. Genet."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1002\/cm.970080110","article-title":"Bending patterns of Chlamydomonas flagella: IV. Mutants with defects in inner and outer dynein arms indicate differences in dynein arm function","volume":"8","author":"Brokaw","year":"1987","journal-title":"Cell Motil. Cytoskelet."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1343","DOI":"10.1164\/rccm.200411-1583OC","article-title":"Mislocalization of DNAH5 and DNAH9 in respiratory cells from patients with primary ciliary dyskinesia","volume":"171","author":"Fliegauf","year":"2005","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1165\/rcmb.2015-0353OC","article-title":"DNAH11 Localization in the Proximal Region of Respiratory Cilia Defines Distinct Outer Dynein Arm Complexes","volume":"55","author":"Dougherty","year":"2016","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"995","DOI":"10.1016\/j.ajhg.2018.10.020","article-title":"Recessive DNAH9 Loss-of-Function Mutations Cause Laterality Defects and Subtle Respiratory Ciliary-Beating Defects","volume":"103","author":"Loges","year":"2018","journal-title":"Am. J. Hum. Genet."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1016\/S2213-2600(19)30374-1","article-title":"Primary ciliary dyskinesia in the genomics age","volume":"8","author":"Lucas","year":"2020","journal-title":"Lancet Respir. Med."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/j.jpeds.2020.05.052","article-title":"Clinical and Genetic Spectrum of Children with Primary Ciliary Dyskinesia in China","volume":"225","author":"Guo","year":"2020","journal-title":"J. Pediatr."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1768","DOI":"10.1016\/j.chest.2021.02.006","article-title":"Clinical and Genetic Spectrum of Children with Primary Ciliary Dyskinesia in China","volume":"159","author":"Guan","year":"2021","journal-title":"Chest"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1186\/s13023-021-01840-2","article-title":"Clinical characteristics and genetic spectrum of 26 individuals of Chinese origin with primary ciliary dyskinesia","volume":"16","author":"Zhao","year":"2021","journal-title":"Orphanet. J. Rare Dis."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"e002686","DOI":"10.1161\/CIRCGEN.119.002686","article-title":"Randomization of Left-Right Asymmetry and Congenital Heart Defects","volume":"12","author":"Wallmeier","year":"2019","journal-title":"Circ. Genom. Precis. Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1038\/s41572-020-0209-6","article-title":"Motile ciliopathies","volume":"6","author":"Wallmeier","year":"2020","journal-title":"Nat. Rev. Dis. Primers"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1038\/cmi.2017.118","article-title":"Chemoattractants and cytokines in primary ciliary dyskinesia and cystic fibrosis: Key players in chronic respiratory diseases","volume":"15","author":"Cockx","year":"2018","journal-title":"Cell Mol. Immunol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"14657","DOI":"10.1038\/s41598-017-15027-y","article-title":"Monocytes from patients with Primary Ciliary Dyskinesia show enhanced inflammatory properties and produce higher levels of pro-inflammatory cytokines","volume":"7","author":"Cockx","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1038\/s41419-022-05010-5","article-title":"Dnah9 mutant mice and organoid models recapitulate the clinical features of patients with PCD and provide an excellent platform for drug screening","volume":"13","author":"Zheng","year":"2022","journal-title":"Cell Death Dis."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1186\/s12964-021-00740-z","article-title":"Long-term differentiating primary human airway epithelial cell cultures: How far are we?","volume":"19","year":"2021","journal-title":"Cell Commun. Signal."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"101848","DOI":"10.1016\/j.scr.2020.101848","article-title":"Generation of two human induced pluripotent stem cell lines (MHHi017-A, MHHi017-B) from a patient with primary ciliary dyskinesia carrying a homozygous mutation (c.7915C > T [p.Arg2639*]) in the DNAH5 gene","volume":"46","author":"Drick","year":"2020","journal-title":"Stem Cell Res."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"66","DOI":"10.3109\/00016489509133349","article-title":"Normal ciliary beat frequency after ciliogenesis in nasal epithelial cells cultured sequentially as monolayer and in suspension","volume":"115","author":"Jorissen","year":"1995","journal-title":"Acta Otolaryngol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1259","DOI":"10.1016\/j.jaci.2012.01.073","article-title":"An ex vivo model of severe asthma using reconstituted human bronchial epithelium","volume":"129","author":"Gras","year":"2012","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1080\/00016480410016270","article-title":"The secondary nature of ciliary (dis)orientation in secondary and primary ciliary dyskinesia","volume":"124","author":"Jorissen","year":"2004","journal-title":"Acta Otolaryngol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e52058","DOI":"10.15252\/embr.202052058","article-title":"Modelling of primary ciliary dyskinesia using patient-derived airway organoids","volume":"22","author":"Bottinger","year":"2021","journal-title":"EMBO Rep."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1136\/archdischild-2017-312902","article-title":"European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia: A guideline review","volume":"104","author":"Dalrymple","year":"2019","journal-title":"Arch. Dis. Child Educ. Pract. Ed."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.jviromet.2004.02.020","article-title":"A simplified plaque assay for respiratory syncytial virus--direct visualization of plaques without immunostaining","volume":"120","year":"2004","journal-title":"J. Virol. Methods"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"dev.177428","DOI":"10.1242\/dev.177428","article-title":"Novel dynamics of human mucociliary differentiation revealed by single-cell RNA sequencing of nasal epithelial cultures","volume":"146","author":"Deprez","year":"2019","journal-title":"Development"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1016\/j.celrep.2015.04.025","article-title":"TGF-beta Signaling Regulates the Differentiation of Motile Cilia","volume":"11","author":"Tozser","year":"2015","journal-title":"Cell Rep."},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Glaser, L., Coulter, P.J., Shields, M., Touzelet, O., Power, U.F., and Broadbent, L. (2019). Airway Epithelial Derived Cytokines and Chemokines and Their Role in the Immune Response to Respiratory Syncytial Virus Infection. Pathogens, 8.","DOI":"10.3390\/pathogens8030106"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.stemcr.2015.11.010","article-title":"Directed Induction of Functional Multi-ciliated Cells in Proximal Airway Epithelial Spheroids from Human Pluripotent Stem Cells","volume":"6","author":"Konishi","year":"2016","journal-title":"Stem Cell Rep."},{"key":"ref_29","first-page":"764","article-title":"BMP signaling and cellular dynamics during regeneration of airway epithelium from basal progenitors","volume":"143","author":"Tadokoro","year":"2016","journal-title":"Development"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1753","DOI":"10.21037\/jtd.2018.02.13","article-title":"Aberrant epithelial remodeling with impairment of cilia architecture in non-cystic fibrosis bronchiectasis","volume":"10","author":"Chen","year":"2018","journal-title":"J. Thorac. Dis."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1513\/pats.200605-126SF","article-title":"Airway epithelial repair, regeneration, and remodeling after injury in chronic obstructive pulmonary disease","volume":"3","author":"Puchelle","year":"2006","journal-title":"Proc. Am. Thorac. Soc."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1513\/pats.200801-010AW","article-title":"Epithelial cell-extracellular matrix interactions and stem cells in airway epithelial regeneration","volume":"5","author":"Coraux","year":"2008","journal-title":"Proc. Am. Thorac. Soc."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1038\/s41577-020-0331-4","article-title":"Pathological inflammation in patients with COVID-19: A key role for monocytes and macrophages","volume":"20","author":"Merad","year":"2020","journal-title":"Nat. Rev. Immunol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1378\/chest.129.1.118","article-title":"Mucus properties in children with primary ciliary dyskinesia: Comparison with cystic fibrosis","volume":"129","author":"Bush","year":"2006","journal-title":"Chest"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1183\/13993003.01390-2015","article-title":"Changes in airway inflammation during pulmonary exacerbations in patients with cystic fibrosis and primary ciliary dyskinesia","volume":"47","author":"Ratjen","year":"2016","journal-title":"Eur. Respir. J."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1016\/j.freeradbiomed.2017.02.038","article-title":"Dipeptide HCH6-1 inhibits neutrophil activation and protects against acute lung injury by blocking FPR1","volume":"106","author":"Yang","year":"2017","journal-title":"Free Radic. Biol. Med."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"812","DOI":"10.1164\/rccm.202005-1583OC","article-title":"Characterization of the Inflammatory Response to Severe COVID-19 Illness","volume":"202","author":"McElvaney","year":"2020","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"108517","DOI":"10.1016\/j.clim.2020.108517","article-title":"Inhibition of cytokine signaling by ruxolitinib and implications for COVID-19 treatment","volume":"218","author":"Yeleswaram","year":"2020","journal-title":"Clin. Immunol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1016\/j.stem.2015.01.002","article-title":"Injury Induces Direct Lineage Segregation of Functionally Distinct Airway Basal Stem\/Progenitor Cell Subpopulations","volume":"16","author":"Law","year":"2015","journal-title":"Cell Stem Cell"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"3338","DOI":"10.1016\/j.celrep.2019.08.063","article-title":"\u0394N-Tp63 Mediates Wnt\/beta-Catenin-Induced Inhibition of Differentiation in Basal Stem Cells of Mucociliary Epithelia","volume":"28","author":"Haas","year":"2019","journal-title":"Cell Rep."}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/11\/24\/4013\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:39:29Z","timestamp":1760146769000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/11\/24\/4013"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,12]]},"references-count":40,"journal-issue":{"issue":"24","published-online":{"date-parts":[[2022,12]]}},"alternative-id":["cells11244013"],"URL":"https:\/\/doi.org\/10.3390\/cells11244013","relation":{},"ISSN":["2073-4409"],"issn-type":[{"value":"2073-4409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,12,12]]}}}