{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T06:04:24Z","timestamp":1787205864547,"version":"build-2736575974"},"reference-count":24,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2024,2,2]],"date-time":"2024-02-02T00:00:00Z","timestamp":1706832000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Sociedad Espa\u00f1ola de Neumolog\u00eda Pedi\u00e1trica","award":["2014"],"award-info":[{"award-number":["2014"]}]},{"name":"Sociedad Espa\u00f1ola de Neumolog\u00eda Pedi\u00e1trica","award":["2015"],"award-info":[{"award-number":["2015"]}]},{"name":"Sociedad Espa\u00f1ola de Neumolog\u00eda Pedi\u00e1trica","award":["OP ERDF of Comunitat Valenciana 2014\u20132020"],"award-info":[{"award-number":["OP ERDF of Comunitat Valenciana 2014\u20132020"]}]},{"name":"Sociedad Valenciana de Neumolog\u00eda","award":["2014"],"award-info":[{"award-number":["2014"]}]},{"name":"Sociedad Valenciana de Neumolog\u00eda","award":["2015"],"award-info":[{"award-number":["2015"]}]},{"name":"Sociedad Valenciana de Neumolog\u00eda","award":["OP ERDF of Comunitat Valenciana 2014\u20132020"],"award-info":[{"award-number":["OP ERDF of Comunitat Valenciana 2014\u20132020"]}]},{"name":"Generalitat Valenciana","award":["2014"],"award-info":[{"award-number":["2014"]}]},{"name":"Generalitat Valenciana","award":["2015"],"award-info":[{"award-number":["2015"]}]},{"name":"Generalitat Valenciana","award":["OP ERDF of Comunitat Valenciana 2014\u20132020"],"award-info":[{"award-number":["OP ERDF of Comunitat Valenciana 2014\u20132020"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Antioxidants"],"abstract":"<jats:p>Background: Primary Ciliary Dyskinesia (PCD) represents a rare condition marked by an abnormal mobility pattern of cilia and flagella, resulting in impaired mucociliary clearance. This deficiency leads to recurrent infections and persistent inflammation of the airways. While previous studies have indicated heightened oxidative stress levels in the exhaled breath condensate of pediatric PCD patients, the assessment of oxidative stress within the affected respiratory tissue remains unexplored. Aims: To assess the oxidative status of human nasal epithelial cells (NECs) in PCD patients. Methods: Thirty-five PCD patients and thirty-five healthy control subjects were prospectively included in the study. Levels of reactive oxygen species (ROS), reactive nitrogen species (RNS), glutathione (GSH), intracellular Ca2+, plasma membrane potential, and oxidative damage in lipids and proteins were measured. In addition, apoptosis and mitochondrial function were analyzed by flow cytometry in NECs. Results: NECs from PCD patients showed reduced levels of apoptosis (p = 0.004), superoxide anion (O2\u2212, p = 0.018), peroxynitrite (ONOO\u2212, p = 0.007), nitric oxide (NO, p = 0.007), mitochondrial hydrogen peroxide (mtH2O2, p &lt; 0.0001), and mitochondrial superoxide anion (mtO2\u2212, p = 0.0004) and increased mitochondrial mass (p = 0.009) compared to those from healthy individuals. No significant differences were observed in oxidized proteins (p = 0.137) and the oxidized\/reduced lipid ratio (p = 0.7973). The oxidative profile of NEC cells in PCD patients, according to their ciliary motility, recurrent otitis, recurrent pneumonia, atelectasis, bronchiectasis, and situs inversus, showed no statistically significant differences in the parameters studied. Conversely, patients with chronic rhinosinusitis exhibited lower levels of ONOO\u2212 than PCD patients without this condition, with no significant differences related to other symptoms. Conclusions: Our findings strongly suggest the presence of a redox imbalance, specifically leaning toward a reductive state, in PCD patients.<\/jats:p>","DOI":"10.3390\/antiox13020190","type":"journal-article","created":{"date-parts":[[2024,2,2]],"date-time":"2024-02-02T09:24:08Z","timestamp":1706865848000},"page":"190","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Redox Imbalance in Nasal Epithelial Cells of Primary Ciliary Dyskinesia Patients"],"prefix":"10.3390","volume":"13","author":[{"given":"Ana","family":"Reula","sequence":"first","affiliation":[{"name":"Valencia University Clinical Hospital Research Foundation, Instituto de Investigaci\u00f3n Sanitaria INCLIVA, Avda. Men\u00e9ndez y Pelayo, 4, 46010 Valencia, Spain"},{"name":"Rare Respiratory Diseases Research Group, Department of Physiology, School of Medicine, University of Valencia, Avda. Blasco Ib\u00e1\u00f1ez, 17, 46010 Valencia, Spain"},{"name":"Biomedical Sciences Department, CEU-Cardenal Herrera University, 12006 Castell\u00f3n, Spain"},{"name":"Molecular, Cellular, and Genomic Biomedicine Group, IIS La Fe, 46026 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0121-2676","authenticated-orcid":false,"given":"Silvia","family":"Castillo-Corull\u00f3n","sequence":"additional","affiliation":[{"name":"Valencia University Clinical Hospital Research Foundation, Instituto de Investigaci\u00f3n Sanitaria INCLIVA, Avda. Men\u00e9ndez y Pelayo, 4, 46010 Valencia, Spain"},{"name":"Paediatrics Unit, Department of Pediatrics, Obstetrics and Gynecology, Hospital Cl\u00ednico Universitario Valencia, University of Valencia, 46022 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8258-6292","authenticated-orcid":false,"given":"Miguel","family":"Armengot","sequence":"additional","affiliation":[{"name":"Molecular, Cellular, and Genomic Biomedicine Group, IIS La Fe, 46026 Valencia, Spain"},{"name":"ENT Unit, Department of Surgery, School of Medicine, Hospital La Fe, University of Valencia, Avda. Blasco Ib\u00e1\u00f1ez, 17, 46010 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guadalupe","family":"Herrera","sequence":"additional","affiliation":[{"name":"Flow Cytometry Unit, Fundaci\u00f3n Investigaci\u00f3n Hospital Cl\u00ednico Valencia, Instituto de Investigaci\u00f3n Sanitaria INCLIVA, University of Valencia, Avda. Men\u00e9ndez y Pelayo, 4, 46010 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amparo","family":"Escribano","sequence":"additional","affiliation":[{"name":"Valencia University Clinical Hospital Research Foundation, Instituto de Investigaci\u00f3n Sanitaria INCLIVA, Avda. Men\u00e9ndez y Pelayo, 4, 46010 Valencia, Spain"},{"name":"Paediatrics Unit, Department of Pediatrics, Obstetrics and Gynecology, Hospital Cl\u00ednico Universitario Valencia, University of Valencia, 46022 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2938-2965","authenticated-orcid":false,"given":"Francisco","family":"Das\u00ed","sequence":"additional","affiliation":[{"name":"Valencia University Clinical Hospital Research Foundation, Instituto de Investigaci\u00f3n Sanitaria INCLIVA, Avda. Men\u00e9ndez y Pelayo, 4, 46010 Valencia, Spain"},{"name":"Rare Respiratory Diseases Research Group, Department of Physiology, School of Medicine, University of Valencia, Avda. Blasco Ib\u00e1\u00f1ez, 17, 46010 Valencia, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,2,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1016\/S2213-2600(21)00453-7","article-title":"The Global Prevalence and Ethnic Heterogeneity of Primary Ciliary Dyskinesia Gene Variants: A Genetic Database Analysis","volume":"10","author":"Hannah","year":"2022","journal-title":"Lancet Respir. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.jpeds.2020.11.040","article-title":"Understanding Primary Ciliary Dyskinesia and Other Ciliopathies","volume":"230","author":"Horani","year":"2021","journal-title":"J. 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