Redox Imbalance in Nasal Epithelial Cells of Primary Ciliary Dyskinesia Patients

被引:0
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作者
Reula, Ana [1 ,2 ,3 ,4 ]
Castillo-Corullon, Silvia [1 ,5 ]
Armengot, Miguel [4 ,6 ]
Herrera, Guadalupe [7 ]
Escribano, Amparo [1 ,5 ]
Dasi, Francisco [1 ,2 ]
机构
[1] Univ Valencia, Inst Invest Sanitaria INCLIVA, Clin Hosp, Res Fdn, Avda Menendez y Pelayo 4, Valencia 46010, Spain
[2] Univ Valencia, Sch Med, Dept Physiol, Res Grp Rare Resp Dis, Avda Blasco Ibanez 15, Valencia 46010, Spain
[3] CEU Cardenal Herrera Univ, Biomed Sci Dept, Castellon de La Plana 12006, Spain
[4] IIS La Fe, Mol Cellular & Genom Biomed Grp, Valencia 46026, Spain
[5] Univ Valencia, Hosp Clin Univ Valencia, Dept Pediat Obstet & Gynecol, Paediat Unit, Valencia 46022, Spain
[6] Univ Valencia, Hosp La Fe, Sch Med, Dept Surg,ENT Unit, Avda Blasco Ibanez 17, Valencia 46010, Spain
[7] Univ Valencia, Fdn Invest Hosp Clin Valencia, Flow Cytometry Unit, Inst Invest Sanitaria INCLIVA, Avda Menendez y Pelayo 4, Valencia 46010, Spain
关键词
primary ciliary dyskinesia; rare respiratory diseases; oxidative stress; nitric oxide; hydrogen peroxide; PEROXYNITRITE INHIBITORY-ACTIVITY; OXIDATIVE STRESS; GLUTATHIONE; CHILDREN; DISEASE;
D O I
10.3390/antiox13020190
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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 (O-2(-), p = 0.018), peroxynitrite (ONOO-, p = 0.007), nitric oxide (NO, p = 0.007), mitochondrial hydrogen peroxide (mtH(2)O(2), p < 0.0001), and mitochondrial superoxide anion (mtO(2)(-), 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- 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.
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页数:13
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