Airway epithelium dysfunction in cystic fibrosis and COPD

Mediators of Inflammation

Volumn 2018, Issue , 2018, Pages

  De Rose, Virginia ^a   Molloy, Kevin ^b   Gohy, Sophie ^c,d   Pilette, Charles ^c,d   Greene, Catherine M ^b  

^a UNIVERSITY OF TURIN   (Italy)

^b BEAUMONT HOSPITAL   (Ireland)

^c UNIVERSITÉ CATHOLIQUE DE LOUVAIN   (Belgium)

^d CLINIQUES UNIVERSITAIRES SAINT LUC   (Belgium)

Author keywords

[No Author keywords available]

Indexed keywords

ANIMAL; CHRONIC OBSTRUCTIVE LUNG DISEASE; CYSTIC FIBROSIS; EPITHELIUM CELL; HUMAN; IMMUNOLOGY; INFLAMMATION; PATHOLOGY;

ANIMALS; CYSTIC FIBROSIS; EPITHELIAL CELLS; HUMANS; INFLAMMATION; PULMONARY DISEASE, CHRONIC OBSTRUCTIVE;

EID: 85053185850     PISSN: 09629351     EISSN: 14661861     Source Type: Journal    
DOI: 10.1155/2018/1309746     Document Type: Article

References (244)

1. F. Ratjen and G. Döring, "Cystic fibrosis," The Lancet, vol. 361, no. 9358, pp. 681-689, 2003.
2. J. S. Elborn, "Cystic fibrosis," The Lancet, vol. 388, no. 10059, pp. 2519-2531, 2016.
3. M. Decramer, W. Janssens, and M. Miravitlles, "Chronic obstructive pulmonary disease," The Lancet, vol. 379, no. 9823, pp. 1341-1351, 2012.
4. P. J. Barnes, P. G. J. Burney, E. K. Silverman et al., "Chronic obstructive pulmonary disease," Nature Reviews Disease Primers, vol. 1, article 15076, 2015.
5. W. Gao, L. Li, Y. Wang et al., "Bronchial epithelial cells: the key effector cells in the pathogenesis of chronic obstructive pulmonary disease," Respirology, vol. 20, no. 5, pp. 722-729, 2015.
6. A. Tam, S. Wadsworth, D. Dorscheid, S. F. P. Man, and D. D. Sin, "The airway epithelium: more than just a structural barrier," Therapeutic Advances in Respiratory Disease, vol. 5, no. 4, pp. 255-273, 2011.
7. S. Ganesan, A. T. Comstock, and U. S. Sajjan, "Barrier function of airway tract epithelium," Tissue Barriers, vol. 1, no. 4, article e24997, 2013.
8. M. Goodwin and A. S. Yap, "Classical cadherin adhesion molecules: coordinating cell adhesion, signaling and the cytoskeleton," Journal of Molecular Histology, vol. 35, no. 8-9, pp. 839-844, 2004.
9. Q. Wang and B. Margolis, "Apical junctional complexes and cell polarity," Kidney International, vol. 72, no. 12, pp. 1448-1458, 2007.
10. E. N. Worthington and R. Tarran, "Methods for ASL measurements and mucus transport rates in cell cultures," Methods in Molecular Biology, vol. 742, pp. 77-92, 2011.
11. L. Tengroth, C. R. Millrud, A. M. Kvarnhammar, S. Kumlien Georén, L. Latif, and L. O. Cardell, "Functional effects of tolllike receptor (TLR)3, 7, 9, RIG-I and MDA-5 stimulation in nasal epithelial cells," PLoS One, vol. 9, no. 6, article e98239, 2014.
12. H. J. Kim, C.-H. Kim, M.-J. Kim et al., "The induction of pattern-recognition receptor expression against influenza a virus through Duox2-derived reactive oxygen species in nasal mucosa," American Journal of Respiratory Cell and Molecular Biology, vol. 53, no. 4, pp. 525-535, 2015.
13. C. Tabeling, H. Scheer, S. M. Schönrock et al., "Nucleotide oligomerization domain 1 ligation suppressed murine allergen-specific T-cell proliferation and airway hyperresponsiveness," American Journal of Respiratory Cell and Molecular Biology, vol. 50, no. 5, pp. 903-911, 2014.
14. J. Bogefors, C. Rydberg, R. Uddman et al., "Nod1, Nod2 and Nalp3 receptors, new potential targets in treatment of allergic rhinitis," Allergy, vol. 65, no. 10, pp. 1222-1226, 2010.
15. J. A. Hirota, M. J. Gold, P. R. Hiebert et al., "The nucleotidebinding domain, leucine-rich repeat protein 3 inflammasome/ IL-1 receptor I axis mediates innate, but not adaptive, immune responses after exposure to particulate matter under 10 m," American Journal of Respiratory Cell and Molecular Biology, vol. 52, no. 1, pp. 96-105, 2015.
16. A. T. Nathan, E. A. Peterson, J. Chakir, and M. Wills-Karp, "Innate immune responses of airway epithelium to house dust mite are mediated through-glucan-dependent pathways," Journal of Allergy and Clinical Immunology, vol. 123, no. 3, pp. 612-618, 2009.
17. B. Mirkovi, M. A. Murray, G. M. Lavelle et al., "The role of short-chain fatty acids, produced by anaerobic bacteria, in the cystic fibrosis airway," American Journal of Respiratory and Critical Care Medicine, vol. 192, no. 11, pp. 1314-1324, 2015.
18. R. J. Lee and N. A. Cohen, "Bitter and sweet taste receptors in the respiratory epithelium in health and disease," Journal of Molecular Medicine, vol. 92, no. 12, pp. 1235-1244, 2014.
19. R. J. Lee and N. A. Cohen, "The emerging role of the bitter taste receptor T2R38 in upper respiratory infection and chronic rhinosinusitis," American Journal of Rhinology & Allergy, vol. 27, no. 4, pp. 283-286, 2013.
20. T. Chirkova, S. Lin, A. G. P. Oomens et al., "CX3CR1 is an important surface molecule for respiratory syncytial virus infection in human airway epithelial cells," Journal of General Virology, vol. 96, no. 9, pp. 2543-2556, 2015.
21. M. W. Turner, "Mannose-binding lectin (MBL) in health and disease," Immunobiology, vol. 199, no. 2, pp. 327-339, 1998.
22. J. R. Wright and D. C. Youmans, "Pulmonary surfactant protein a stimulates chemotaxis of alveolar macrophage," American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 264, no. 4, pp. L338-L344, 1993.
23. P. J. Borron, E. A. Mostaghel, C. Doyle, E. S. Walsh, M. G. McHeyzer-Williams, and J. R. Wright, "Pulmonary surfactant proteins A and D directly suppress CD3+/CD4+ cell function: evidence for two shared mechanisms," The Journal of Immunology, vol. 169, no. 10, pp. 5844-5850, 2002.
24. P. H. Pandya and D. S. Wilkes, "Complement system in lung disease," American Journal of Respiratory Cell and Molecular Biology, vol. 51, no. 4, pp. 467-473, 2014.
25. C. M. Greene, S. J. Marciniak, J. Teckman et al., "1-Antitrypsin deficiency," Nature Reviews Disease Primers, vol. 2, article 16051, 2016.
26. R. C. Thompson and K. Ohlsson, "Isolation, properties, and complete amino acid sequence of human secretory leukocyte protease inhibitor, a potent inhibitor of leukocyte elastase," Proceedings of the National Academy of Sciences, vol. 83, no. 18, pp. 6692-6696, 1986.
27. O. Wiedow, J. Harder, J. Bartels, V. Streit, and E. Christophers, "Antileukoprotease in human skin: an antibiotic peptide constitutively produced by keratinocytes," Biochemical and Biophysical Research Communications, vol. 248, no. 3, pp. 904-909, 1998.
28. J. M. Sallenave and A. Silva, "Characterization and gene sequence of the precursor of elafin, an elastase-specific inhibitor in bronchial secretions," American Journal of Respiratory Cell and Molecular Biology, vol. 8, no. 4, pp. 439-445, 1993.
29. J. W. McMichael, A. Roghanian, L. Jiang, R. Ramage, and J.-M. Sallenave, "The antimicrobial antiproteinase elafin binds to lipopolysaccharide and modulates macrophage responses," American Journal of Respiratory Cell and Molecular Biology, vol. 32, no. 5, pp. 443-452, 2005.
30. K. Baranger, M. L. Zani, J. Chandenier, S. Dallet-Choisy, and T. Moreau, "The antibacterial and antifungal properties of trappin-2 (pre-elafin) do not depend on Its protease inhibitory function," FEBS Journal, vol. 275, no. 9, pp. 2008-20, 2008.
31. T. Ganz, "Antimicrobial polypeptides in host defense of the respiratory tract," Journal of Clinical Investigation, vol. 109, no. 6, pp. 693-697, 2002.
32. A. O. Qamruddin, M. A. Alkawash, and J. S. Soothill, "Antibiotic susceptibility of Stenotrophomonas maltophilia in the presence of lactoferrin," Antimicrobial Agents and Chemotherapy, vol. 49, no. 10, pp. 4425-4426, 2005.
33. O. E. Sørensen, P. Follin, A. H. Johnsen et al., "Human cathelicidin, hCAP-18, is processed to the antimicrobial peptide LL-37 by extracellular cleavage with proteinase 3," Blood, vol. 97, no. 12, pp. 3951-3959, 2001.
34. J.-R. C. García, A. Krause, S. Schulz et al., "Human-defensin 4: a novel inducible peptide with a specific salt-sensitive spectrum of antimicrobial activity," The FASEB Journal, vol. 15, no. 10, pp. 1819-1821, 2001.
35. Z. Feng, G. R. Dubyak, M. M. Lederman, and A. Weinberg, "Cutting edge: human defensin 3-a novel antagonist of the HIV-1 coreceptor CXCR4," The Journal of Immunology, vol. 177, no. 2, pp. 782-786, 2006.
36. J. Tomalka, E. Azodi, H. P. Narra et al., "-Defensin 1 plays a role in acute mucosal defense against Candida albicans," The Journal of Immunology, vol. 194, no. 4, pp. 1788-1795, 2015.
37. F. Niyonsaba, H. Ogawa, and I. Nagaoka, "Human-defensin-2 functions as a chemotactic agent for tumour necrosis factor-treated human neutrophils," Immunology, vol. 111, no. 3, pp. 273-281, 2004.
38. J. V. Fahy and B. F. Dickey, "Airway mucus function and dysfunction," New England Journal of Medicine, vol. 363, no. 23, pp. 2233-2247, 2010.
39. C. Martin, J. Frija-Masson, and P.-R. Burgel, "Targeting mucus hypersecretion: new therapeutic opportunities for COPD," Drugs, vol. 74, no. 10, pp. 1073-1089, 2014.
40. M. C. Rose and J. A. Voynow, "Respiratory tract mucin genes and mucin glycoproteins in health and disease," Physiological Reviews, vol. 86, no. 1, pp. 245-278, 2006.
41. M. G. Roy, A. Livraghi-Butrico, A. A. Fletcher et al., "Muc5b is required for airway defence," Nature, vol. 505, no. 7483, pp. 412-416, 2014.
42. A. Ghosh, R. C. Boucher, and R. Tarran, "Airway hydration and COPD," Cellular and Molecular Life Sciences, vol. 72, no. 19, pp. 3637-3652, 2015.
43. S. Blouquit-Laye and T. Chinet, "Ion and liquid transport across the bronchiolar epithelium," Respiratory Physiology & Neurobiology, vol. 159, no. 3, pp. 278-282, 2007.
44. M. Mall, B. R. Grubb, J. R. Harkema, W. K. O'Neal, and R. C. Boucher, "Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice," Nature Medicine, vol. 10, no. 5, pp. 487-493, 2004.
45. Z. Zhou, J. Duerr, B. Johannesson et al., "The ENaCoverexpressing mouse as a model of cystic fibrosis lung disease," Journal of Cystic Fibrosis, vol. 10, no. 2, pp. S172-S182, 2011.
46. M.A. Mall, J. R.Harkema, J. B. Trojanek et al., "Developmentof chronic bronchitis and emphysema in-epithelial Na+ channel-overexpressing mice," American Journal of Respiratory and Critical Care Medicine, vol. 177, no. 7, pp. 730-742, 2008.
47. P. Anagnostopoulou, B. Riederer, J. Duerr et al., "SLC26A9-mediated chloride secretion prevents mucus obstruction in airway inflammation," Journal of Clinical Investigation, vol. 122, no. 10, pp. 3629-3634, 2012.
48. S. Gehrig, J. Duerr, M. Weitnauer et al., "Lack of neutrophil elastase reduces inflammation, mucus hypersecretion, and emphysema, but not mucus obstruction, in mice with cystic fibrosis-like lung disease," American Journal of Respiratory and Critical Care Medicine, vol. 189, no. 9, pp. 1082-1092, 2014.
49. B. Button, L. H. Cai, C. Ehre et al., "A periciliary brush promotes the lung health by separating the mucus layer from airway epithelia," Science, vol. 337, no. 6097, pp. 937-941, 2012.
50. N. Derichs, B. J. Jin, Y. Song, W. E. Finkbeiner, and A. S. Verkman, "Hyperviscous airway periciliary and mucous liquid layers in cystic fibrosis measured by confocal fluorescence photobleaching," The FASEB Journal, vol. 25, no. 7, pp. 2325-2332, 2011.
51. X. X. Tang, L. S. Ostedgaard, M. J. Hoegger et al., "Acidic pH increases airway surface liquid viscosity in cystic fibrosis," Journal of Clinical Investigation, vol. 126, no. 3, pp. 879-891, 2016.
52. A. A. Pezzulo, X. X. Tang, M. J. Hoegger et al., "Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung," Nature, vol. 487, no. 7405, pp. 109-113, 2012.
53. V. S. Shah, D. K. Meyerholz, X. X. Tang et al., "Airway acidification initiates host defense abnormalities in cystic fibrosis mice," Science, vol. 351, no. 6272, pp. 503-507, 2016.
54. M. J. Welsh, "Cigarette smoke inhibition of ion transport in canine tracheal epithelium," Journal of Clinical Investigation, vol. 71, no. 6, pp. 1614-1623, 1983.
55. A. M. Cantin, J. W. Hanrahan, G. Bilodeau et al., "Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers," American Journal of Respiratory and Critical Care Medicine, vol. 173, no. 10, pp. 1139-1144, 2006.
56. M. T. Dransfield, A. M. Wilhelm, B. Flanagan et al., "Acquired cystic fibrosis transmembrane conductance regulator dysfunction in the lower airways in COPD," Chest, vol. 144, no. 2, pp. 498-506, 2013.
57. J. L. Kreindler, A. D. Jackson, P. A. Kemp, R. J. Bridges, and H. Danahay, "Inhibition of chloride secretion in human bronchial epithelial cells by cigarette smoke extract," American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 288, no. 5, pp. L894-L902, 2005.
58. L. A. Clunes, C. M. Davies, R. D. Coakley et al., "Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration," The FASEB Journal, vol. 26, no. 2, pp. 533-545, 2012.
59. P. A. Sloane, S. Shastry, A. Wilhelm et al., "A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease," PLoS One, vol. 7, no. 6, p. e39809, 2012.
60. M. A. Mall, "Unplugging mucus in cystic fibrosis and chronic obstructive pulmonary disease," Annals of the American Thoracic Society, vol. 13, Supplement 2, pp. S177-S185, 2016.
61. J. E. Rasmussen, J. T. Sheridan, W. Polk, C. M. Davies, and R. Tarran, "Cigarette smoke-induced Ca2+ release leads to cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction," Journal of Biological Chemistry, vol. 289, no. 11, pp. 7671-7681, 2014.
62. E. Gensch, M. Gallup, A. Sucher et al., "Tobacco smoke control of mucin production in lung cells requires oxygen radicals AP-1 and JNK," Journal of Biological Chemistry, vol. 279, no. 37, pp. 39085-39093, 2004.
63. Y. A. Mebratu, K. Schwalm, K. R. Smith, M. Schuyler, and Y. Tesfaigzi, "Cigarette smoke suppresses Bik to cause epithelial cell hyperplasia and mucous cell metaplasia," American Journal of Respiratory and Critical Care Medicine, vol. 183, no. 11, pp. 1531-1538, 2011.
64. J. S. Zhou, Y. Zhao, H. B. Zhou et al., "Autophagy plays an essential role in cigarette smoke-induced expression of MUC5AC in airway epithelium," American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 310, no. 11, pp. L1042-L1052, 2016.
65. S. J. Coles, L. R. Levine, and L. Reid, "Hypersecretion of mucus glycoproteins in rat airways induced by tobacco smoke," American Journal of Pathology, vol. 94, no. 3, pp. 459-472, 1979.
66. D. Lamb and L. Reid, "Goblet cell increase in rat bronchial epithelium after exposure to cigarette and cigar tobacco smoke," BMJ, vol. 1, no. 5635, pp. 33-35, 1969.
67. C. Basbaum, H. Lemjabbar, M. Longphre, D. Li, E. Gensch, and N. McNamara, "Control of mucin transcription by diverse injury-induced signaling pathways," American Journal of Respiratory and Critical Care Medicine, vol. 160, Supplement 1, pp. S44-S48, 1999.
68. M. A. Mall and D. Hartl, "CFTR: cystic fibrosis and beyond," European Respiratory Journal, vol. 44, no. 4, pp. 1042-1054, 2014.
69. J. A. Dye and K. B. Adler, "Effects of cigarette smoke on epithelial cells of the respiratory tract," Thorax, vol. 49, no. 8, pp. 825-834, 1994.
70. J. Rennolds, S. Butler, K. Maloney et al., "Cadmium regulates the expression of the CFTR chloride channel in human airway epithelial cells," Toxicological Sciences, vol. 116, no. 1, pp. 349-358, 2010.
71. S. V. Raju, P. L. Jackson, C. A. Courville et al., "Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function," American Journal of Respiratory and Critical Care Medicine, vol. 188, no. 11, pp. 1321-1330, 2013.
72. A. Yaghi, A. Zaman, G. Cox, and M. B. Dolovich, "Ciliary beating is depressed in nasal cilia from chronic obstructive pulmonary disease subjects," Respiratory Medicine, vol. 106, no. 8, pp. 1139-1147, 2012.
73. A. M. Agius, L. A. Smallman, and A. L. Pahor, "Age, smoking and nasal ciliary beat frequency," Clinical Otolaryngology and Allied Sciences, vol. 23, no. 3, pp. 227-230, 1998.
74. E. Tamashiro, G. Xiong, W. T. Anselmo-Lima, J. L. Kreindler, J. N. Palmer, and N. A. Cohen, "Cigarette smoke exposure impairs respiratory epithelial ciliogenesis," American Journal of Rhinology & Allergy, vol. 23, no. 2, pp. 117-122, 2009.
75. Z. Zhou-Suckow, J. Duerr, M. Hagner, R. Agrawal, and M. A. Mall, "Airway mucus, inflammation and remodeling: emerging links in the pathogenesis of chronic lung diseases," Cell and Tissue Research, vol. 367, no. 3, pp. 537-550, 2017.
76. M. Bodas, T. Min, S. Mazur, and N. Vij, "Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema," The Journal of Immunology, vol. 186, no. 1, pp. 602-613, 2011.
77. M. O. Wielpütz, M. Eichinger, Z. Zhou et al., "In vivo monitoring of cystic fibrosis-like lung disease in mice by volumetric computed tomography," European Respiratory Journal, vol. 38, no. 5, pp. 1060-1070, 2011.
78. M. Galluzzo, E. Ciraolo, M. Lucattelli et al., "Genetic deletion and pharmacological inhibition of PI3K reduces neutrophilic airway inflammation and lung damage in mice with cystic fibrosis-like lung disease," Mediators of Inflammation, vol. 2015, Article ID 545417, 10 pages, 2015.
79. S. T. Ballard, L. Trout, Z. Bebök, E. J. Sorscher, and A. Crews, "CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glands," American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 277, no. 4, pp. L694-L699, 1999.
80. M. J. Hug, T. Tamada, and R. J. Bridges, "CFTR and bicarbonate secretion to epithelial cells," Physiology, vol. 18, no. 1, pp. 38-42, 2003.
81. A. M. Cantin, G. Bilodeau, C. Ouellet, J. Liao, and J. W. Hanrahan, "Oxidant stress suppresses CFTR expression," American Journal of Physiology-Cell Physiology, vol. 290, no. 1, pp. C262-C270, 2006.
82. A. M. Cantin, "Cystic fibrosis transmembrane conductance regulator. Implications in cystic fibrosis and chronic obstructive pulmonary disease," Annals of the American Thoracic Society, Supplement 2, pp. S150-S155, 2016.
83. M. Bodas, D. Silverberg, K. Walworth, K. Brucia, and N. Vij, "Augmentation of S-nitrosoglutathione controls cigarette smoke-induced inflammatory-oxidative stress and chronic obstructive pulmonary disease-emphysema pathogenesis by restoring cystic fibrosis transmembrane conductance regulator function," Antioxidants & Redox Signaling, vol. 27, no. 7, pp. 433-451, 2017.
84. P. S. Hiemstra, P. B. McCray Jr, and R. Bals, "The innate immune function of airway epithelial cells in inflammatory lung disease," European Respiratory Journal, vol. 45, no. 4, pp. 1150-1162, 2015.
85. R. Bals and P. S. Hiemstra, "Innate immunity in the lung: how epithelial cells fight against respiratory pathogens," European Respiratory Journal, vol. 23, no. 2, pp. 327-333, 2004.
86. D. Parker and A. Prince, "Innate immunity in the respiratory epithelium," American Journal of Respiratory Cell and Molecular Biology, vol. 45, no. 2, pp. 189-201, 2011.
87. A. M. Cantin, D. Hartl, M. W. Konstan, and J. F. Chmiel, "Inflammation in cystic fibrosis lung disease: pathogenesis and therapy," Journal of Cystic Fibrosis, vol. 14, no. 4, pp. 419-430, 2015.
88. D. Nichols, J. Chmiel, and M. Berger, "Chronic inflammation in the cystic fibrosis lung: alterations in inter-and intracellular signaling," Clinical Reviews in Allergy & Immunology, vol. 34, no. 2, pp. 146-162, 2008.
89. V. De Rose, "Mechanisms and markers of airway inflammation in cystic fibrosis," European Respiratory Journal, vol. 19, no. 2, pp. 333-340, 2002.
90. M. Cohen-Cymberknoh, E. Kerem, T. Ferkol, and A. Elizur, "Airway inflammation in cystic fibrosis: molecular mechanisms and clinical implications," Thorax, vol. 68, no. 12, pp. 1157-1162, 2013.
91. J. A. Voynow and B. K. Rubin, "Mucins, mucus, and sputum," Chest, vol. 135, no. 2, pp. 505-512, 2009.
92. C. M. Greene, T. P. Carroll, S. G. J. Smith et al., "TLR-induced inflammation in cystic fibrosis and non-cystic fibrosis airway epithelial cells," The Journal of Immunology, vol. 174, no. 3, pp. 1638-1646, 2005.
93. S. H. Chotirmall, C. M. Greene, I. K. Oglesby et al., "17-estradiol inhibits IL-8 in cystic fibrosis by up-regulating secretory leucoprotease inhibitor," American Journal of Respiratory and Critical Care Medicine, vol. 182, no. 1, pp. 62-72, 2010.
94. E. Voss, J. Wehkamp, K. Wehkamp, E. F. Stange, J. M. Schroder, and J. Harder, "NOD2/CARD15 mediates induction of the antimicrobial peptide human beta-defensin-2," Journal of Biological Chemistry, vol. 281, no. 4, pp. 2005-2011, 2006.
95. L. Roussel and S. Rousseau, "IL-17 primes airway epithelial cells lacking functional cystic fibrosis transmembrane conductance regulator (CFTR) to increase NOD1 responses," Biochemical and Biophysical Research Communications, vol. 391, no. 1, pp. 505-509, 2010.
96. M. S. Muhlebach, S. L. MacDonald, B. Button et al., "Association between mannan-binding lectin and impaired lung function in cystic fibrosis may be age-dependent," Clinical and Experimental Immunology, vol. 145, no. 2, pp. 302-307, 2006.
97. A. D. Postle, A. Mander, K. B. M. Reid et al., "Deficient hydrophilic lung surfactant proteins A and D with normal surfactant phospholipid molecular species in cystic fibrosis," American Journal of Respiratory Cell and Molecular Biology, vol. 20, no. 1, pp. 90-98, 1999.
98. T. L. Noah, P. C. Murphy, J. J. Alink et al., "Bronchoalveolar lavage fluid surfactant protein-A and surfactant protein-D are inversely related to inflammation in early cystic fibrosis," American Journal of Respiratory and Critical Care Medicine, vol. 168, no. 6, pp. 685-691, 2003.
99. J. F. Alcorn and J. R. Wright, "Degradation of pulmonary surfactant protein D by Pseudomonas aeruginosa elastase abrogates innate immune function," Journal of Biological Chemistry, vol. 279, no. 29, pp. 30871-30879, 2004.
100. J. L. Malloy, R. A. W. Veldhuizen, B. A. Thibodeaux, R. J. O'Callaghan, and J. R. Wright, "Pseudomonas aeruginosa protease IV degrades surfactant proteins and inhibits surfactant host defense and biophysical functions," American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 288, no. 2, pp. L409-L418, 2005.
101. P. B. Van de Weert-van Leeuwen, M. A. Van Meegen, J. J. Speirs et al., "Optimal complement-mediated phagocytosis of Pseudomonas aeruginosa by monocytes is cystic fibrosis transmembrane conductance regulator-dependent," American Journal of Respiratory Cell and Molecular Biology, vol. 49, no. 3, pp. 463-470, 2013.
102. O. Tabary, J. M. Zahm, J. Hinnrasky et al., "Selective upregulation of chemokine IL-8 expression in cystic fibrosis bronchial gland cells in vivo and in vitro," The American Journal of Pathology, vol. 153, no. 3, pp. 921-930, 1998.
103. M. Tsuchiya, P. Kumar, S. Bhattacharyya et al., "Differential regulation of inflammation by inflammatory mediators in cystic fibrosis lung epithelial cells," Journal of Interferon & Cytokine Research, vol. 33, no. 3, pp. 121-129, 2013.
104. E. Osika, J. M. Cavaillon, K. Chadelat et al., "Distinct sputum cytokine profiles in cystic fibrosis and other chronic inflammatory airway disease," European Respiratory Journal, vol. 14, no. 2, pp. 339-346, 1999.
105. P. S. Salva, N. A. Doyle, L. Graham, H. Eigen, and C. M. Doerschuk, "TNF-, IL-8, soluble ICAM-1, and neutrophils in sputum of cystic fibrosis patients," Pediatric Pulmonology, vol. 21, no. 1, pp. 11-19, 1996.
106. A. Decraene, A. Willems-Widyastuti, A. Kasran, K. De Boeck, D. M. Bullens, and L. J. Dupont, "Elevated expression of both mRNA and protein levels of IL-17A in sputum of stable cystic fibrosis patients," Respiratory Research, vol. 11, no. 1, 2010.
107. M. Brodlie, M. C. McKean, G. E. Johnson et al., "Raised interleukin-17 is immunolocalised to neutrophils in cystic fibrosis lung disease," European Respiratory Journal, vol. 37, no. 6, pp. 1378-1385, 2011.
108. K. Tiringer, A. Treis, P. Fucik et al., "A Th17-and Th2-skewed cytokine profile in cystic fibrosis lungs represents a potential risk factor for Pseudomonas aeruginosa infection," American Journal of Respiratory and Critical Care Medicine, vol. 187, no. 6, pp. 621-629, 2013.
109. C. C. Taggart, G. J. Lowe, C. M. Greene et al., "Cathepsin B, L, and S cleave and inactivate secretory leucoprotease inhibitor," Journal of Biological Chemistry, vol. 276, no. 36, pp. 33345-33352, 2001.
110. N. Guyot, M. W. Butler, P. McNally et al., "Elafin, an elastasespecific inhibitor, is cleaved by its cognate enzyme neutrophil elastase in sputum from individuals with cystic fibrosis," Journal of Biological Chemistry, vol. 283, no. 47, pp. 32377-32385, 2008.
111. P. Valenti, A. Catizone, F. Pantanella et al., "Lactoferrin decreases inflammatory response by cystic fibrosis bronchial cells invaded with Burkholderia cenocepacia iron-modulated biofilm," International Journal of Immunopathology and Pharmacology, vol. 24, no. 4, pp. 1057-1068, 2011.
112. B. E. Britigan, M. B. Hayek, B. N. Doebbeling, and R. B. Fick Jr, "Transferrin and lactoferrin undergo proteolytic cleavage in the Pseudomonas aeruginosa-infected lungs of patients with cystic fibrosis," Infection and Immunity, vol. 61, no. 12, pp. 5049-5055, 1993.
113. M. P. Rogan, C. C. Taggart, C. M. Greene, P. G. Murphy, S. J. O'Neill, and N. G. McElvaney, "Loss of microbicidal activity and increased formation of biofilm due to decreased lactoferrin activity in patients with cystic fibrosis," The Journal of Infectious Diseases, vol. 190, no. 7, pp. 1245-1253, 2004.
114. G. Bergsson, E. P. Reeves, P. McNally et al., "LL-37 complexation with glycosaminoglycans in cystic fibrosis lungs inhibits antimicrobial activity, which can be restored by hypertonic saline," The Journal of Immunology, vol. 183, no. 1, pp. 543-551, 2009.
115. D. J. Weiner, R. Bucki, and P. A. Janmey, "The antimicrobial activity of the cathelicidin LL37 is inhibited by F-actin bundles and restored by gelsolin," American Journal of Respiratory Cell and Molecular Biology, vol. 28, no. 6, pp. 738-745, 2003.
116. R. Tesse, F. Cardinale, T. Santostasi et al., "Association of-defensin-1 gene polymorphisms with Pseudomonas aeruginosa airway colonization in cystic fibrosis," Genes & Immunity, vol. 9, no. 1, pp. 57-60, 2008.
117. C. C. Taggart, C. M. Greene, S. G. Smith et al., "Inactivation of human-defensins 2 and 3 by elastolytic cathepsins," The Journal of Immunology, vol. 171, no. 2, pp. 931-937, 2003.
118. T. Yoshida and R. M. Tuder, "Pathobiology of cigarette smoke-induced chronic obstructive pulmonary disease," Physiological Reviews, vol. 87, no. 3, pp. 1047-1082, 2007.
119. P. J. Barnes, "Immunology of asthma and chronic obstructive pulmonary disease," Nature Reviews Immunology, vol. 8, no. 3, pp. 183-192, 2008.
120. R. Shaykhiev and R. G. Crystal, "Early events in the pathogenesis of chronic obstructive pulmonary disease. Smoking-induced reprogramming of airway epithelial basal progenitor cells," Annals of the American Thoracic Society, vol. 11, Supplement 5, pp. S252-S258, 2014.
121. S. T. Gohy, C. Hupin, C. Pilette, and M. Z. Ladjemi, "Chronic inflammatory airway diseases: the central role of the epithelium revisited," Clinical & Experimental Allergy, vol. 46, no. 4, pp. 529-542, 2016.
122. J. C. Hogg and W. Timens, "The pathology of chronic obstructive pulmonary disease," Annual Review of Pathology: Mechanisms of Disease, vol. 4, no. 1, pp. 435-459, 2009.
123. F. Hassan, X. Xu, G. Nuovo et al., "Accumulation of metals in GOLD4 COPD lungs is associated with decreased CFTR levels," Respiratory Research, vol. 15, no. 1, p. 69, 2014.
124. I. H. Heijink, S. M. Brandenburg, D. S. Postma, and A. J. M. van Oosterhout, "Cigarette smoke impairs airway epithelial barrier function and cell-cell contact recovery," European Respiratory Journal, vol. 39, no. 2, pp. 419-428, 2012.
125. R. Shaykhiev, F. Otaki, P. Bonsu et al., "Cigarette smoking reprograms apical junctional complex molecular architecture in the human airway epithelium in vivo," Cellular and Molecular Life Sciences, vol. 68, no. 5, pp. 877-892, 2011.
126. J. Milara, T. Peiró, A. Serrano, and J. Cortijo, "Epithelial to mesenchymal transition is increased in patients with COPD and induced by cigarette smoke," Thorax, vol. 68, no. 5, pp. 410-420, 2013.
127. P. L. Leopold, M. J. O'Mahony, X. Julie Lian, A. E. Tilley, B.-G. Harvey, and R. G. Crystal, "Smoking is associated with shortened airway cilia," PLoS ONE, vol. 4, no. 12, article e8157, 2009.
128. S. M. Kennedy, R. K. Elwood, B. J. Wiggs, P. D. Paré, and J. C. Hogg, "Increased airway mucosal permeability of smokers: relationship to airway reactivity," American Review of Respiratory Disease, vol. 129, no. 1, pp. 143-148, 1984.
129. J. Hessel, J. Heldrich, J. Fuller et al., "Intraflagellar transport gene expression associated with short cilia in smoking and COPD," PLoS One, vol. 9, no. 1, article e85453, 2014.
130. E. J. Peters, R. Morice, S. E. Benner et al., "Squamous metaplasia of the bronchial mucosa and its relationship to smoking," Chest, vol. 103, no. 5, pp. 1429-1432, 1993.
131. A. B. Lumsden, A. McLean, and D. Lamb, "Goblet and Clara cells of human distal airways: evidence for smoking induced changes in their numbers," Thorax, vol. 39, no. 11, pp. 844-849, 1984.
132. V. V. Polosukhin, J. M. Cates, W. E. Lawson et al., "Bronchial secretory immunoglobulin a deficiency correlates with airway inflammation and progression of chronic obstructive pulmonary disease," American Journal of Respiratory and Critical Care Medicine, vol. 184, no. 3, pp. 317-327, 2011.
133. T. Nyunoya, Y. Mebratu, A. Contreras, M. Delgado, H. S. Chand, and Y. Tesfaigzi, "Molecular processes that drive cigarette smoke-induced epithelial cell fate of the lung," American Journal of Respiratory Cell and Molecular Biology, vol. 50, no. 3, pp. 471-482, 2014.
134. M. J. Holtzman, D. E. Byers, J. Alexander-Brett, and X. Wang, "The role of airway epithelial cells and innate immune cells in chronic respiratory disease," Nature Reviews Immunology, vol. 14, no. 10, pp. 686-698, 2014.
135. U. S. Sajjan, "Susceptibility to viral infections in chronic obstructive pulmonary disease: role of epithelial cells," Current Opinion in Pulmonary Medicine, vol. 19, no. 2, pp. 125-132, 2013.
136. M. Golec, C. Reichel, B. Mackiewicz et al., "Cathelicidin LL-37, granzymes, TGF-beta1 and cytokines levels in induced sputum from farmers with and without COPD," Annals of Agricultural and Environmental Medicine, vol. 16, no. 2, pp. 289-297, 2009.
137. Y. Y. Jiang, W. Xiao, M. X. Zhu et al., "The effect of human antibacterial peptide LL-37 in the pathogenesis of chronic obstructive pulmonary disease," Respiratory Medicine, vol. 106, no. 12, pp. 1680-1689, 2012.
138. W. Xiao, Y. P. Hsu, A. Ishizaka, T. Kirikae, and R. B. Moss, "Sputum cathelicidin, urokinase plasminogen activation system components, and cytokines discriminate cystic fibrosis, COPD, and asthma inflammation," Chest, vol. 128, no. 4, pp. 2316-2326, 2005.
139. M. Tsoumakidou, I. Bouloukaki, K. Thimaki, N. Tzanakis, and N. M. Siafakas, "Innate immunity proteins in chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis," Experimental Lung Research, vol. 36, no. 6, pp. 373-380, 2010.
140. G. I. Parameswaran, S. Sethi, and T. F. Murphy, "Effects of bacterial infection on airway antimicrobial peptides and proteins in COPD," Chest, vol. 140, no. 3, pp. 611-617, 2011.
141. G. Paone, V. Conti, A. Vestri et al., "Analysis of sputum markers in the evaluation of lung inflammation and functional impairment in symptomatic smokers and COPD patients," Disease Markers, vol. 31, no. 2, pp. 91-100, 2011.
142. D. Merkel, W. Rist, P. Seither, A. Weith, and M. C. Lenter, "Proteomic study of human bronchoalveolar lavage fluids from smokers with chronic obstructive pulmonary disease by combining surface-enhanced laser desorption/ionizationmass spectrometry profiling with mass spectrometric protein identification," Proteomics, vol. 5, no. 11, pp. 2972-2980, 2005.
143. C. I.-U. Chen, S. Schaller-Bals, K. P. Paul, U. Wahn, and R. Bals, "-defensins and LL-37 in bronchoalveolar lavage fluid of patients with cystic fibrosis," Journal of Cystic Fibrosis, vol. 3, no. 1, pp. 45-50, 2004.
144. L. J. P. Persson, M. Aanerud, J. A. Hardie et al., "Antimicrobial peptide levels are linked to airway inflammation, bacterial colonisation and exacerbations in chronic obstructive pulmonary disease," European Respiratory Journal, vol. 49, no. 3, article 1601328, 2017.
145. P. Mallia, J. Footitt, R. Sotero et al., "Rhinovirus infection induces degradation of antimicrobial peptides and secondary bacterial infection in chronic obstructive pulmonary disease," American Journal of Respiratory and Critical Care Medicine, vol. 186, no. 11, pp. 1117-1124, 2012.
146. A. B. Thompson, T. Bohling, F. Payvandi, and S. I. Rennard, "Lower respiratory tract lactoferrin and lysozyme arise primarily in the airways and are elevated in association with chronic bronchitis," The Journal of Laboratory and Clinical Medicine, vol. 115, no. 2, pp. 148-158, 1990.
147. B. L. Luo, R. C. Niu, J. T. Feng, C. P. Hu, X. Y. Xie, and L. J. Ma, "Downregulation of secretory leukocyte proteinase inhibitor in chronic obstructive lung disease: the role of TGF-/Smads signaling pathways," Archives of Medical Research, vol. 39, no. 4, pp. 388-396, 2008.
148. C. Hollander, B. Sitkauskiene, R. Sakalauskas, U. Westin, and S. M. Janciauskiene, "Serum and bronchial lavage fluid concentrations of IL-8, SLPI, sCD14 and sICAM-1 in patients with COPD and asthma," Respiratory Medicine, vol. 101, no. 9, pp. 1947-1953, 2007.
149. E. Pace, A. Giarratano, M. Ferraro et al., "TLR4 upregulation underpins airway neutrophilia in smokers with chronic obstructive pulmonary disease and acute respiratory failure," Human Immunology, vol. 72, no. 1, pp. 54-62, 2011.
150. P. J. Barnes, S. D. Shapiro, and R. A. Pauwels, "Chronic obstructive pulmonary disease: molecular and cellular mechanisms," European Respiratory Journal, vol. 22, no. 4, pp. 672-688, 2003.
151. A. A. Lugade, P. N. Bogner, T. H. Thatcher, P. J. Sime, R. P. Phipps, and Y. Thanavala, "Cigarette smoke exposure exacerbates lung inflammation and compromises immunity to bacterial infection," The Journal of Immunology, vol. 192, no. 11, pp. 5226-5235, 2014.
152. G. G. Brusselle, G. F. Joos, and K. R. Bracke, "New insights into the immunology of chronic obstructive pulmonary disease," The Lancet, vol. 378, no. 9795, pp. 1015-1026, 2011.
153. T. A. Bhat, L. Panzica, S. G. Kalathil, and Y. Thanavala, "Immune dysfunction in patients with chronic obstructive pulmonary disease," Annals of the American Thoracic Society, vol. 12, Supplement 2, pp. S169-S175, 2015.
154. T. Maes, K. R. Bracke, K. Y. Vermaelen et al., "Murine TLR4 is implicated in cigarette smoke-induced pulmonary inflammation," International Archives of Allergy and Immunology, vol. 141, no. 4, pp. 354-368, 2006.
155. N. Ferhani, S. Letuve, A. Kozhich et al., "Expression of highmobility group box 1 and of receptor for advanced glycation end products in chronic obstructive pulmonary disease," American Journal of Respiratory and Critical Care Medicine, vol. 181, no. 9, pp. 917-927, 2010.
156. M. Lommatzsch, S. Cicko, T. Müller et al., "Extracellular adenosine triphosphate and chronic obstructive pulmonary disease," American Journal of Respiratory and Critical Care Medicine, vol. 181, no. 9, pp. 928-934, 2010.
157. R. Y. Kim, J. W. Pinkerton, P. G. Gibson, M. A. Cooper, J. C. Horvat, and P. M. Hansbro, "Inflammasomes in COPD and neutrophilic asthma," Thorax, vol. 70, no. 12, pp. 1199-1201, 2015.
158. P. J. Barnes, "Inflammatory mechanisms in patients with chronic obstructive pulmonary disease," Journal of Allergy and Clinical Immunology, vol. 138, no. 1, pp. 16-27, 2016.
159. A. Churg, S. Zhou, X. Wang, R. Wang, and J. L. Wright, "The role of interleukin-1 in murine cigarette smoke-induced emphysema and small airway remodeling," American Journal of Respiratory Cell and Molecular Biology, vol. 40, no. 4, pp. 482-490, 2009.
160. C. Schulz, K. Wolf, M. Harth, K. Krätzel, L. Kunz-Schughart, and M. Pfeifer, "Expression and release of interleukin-8 by human bronchial epithelial cells from patients with chronic obstructive pulmonary disease, smokers, and neversmokers," Respiration, vol. 70, no. 3, pp. 254-261, 2003.
161. E. F. A. van 't Wout, J. A. Dickens, A. van Schadewijk et al., "Increased ERK signalling promotes inflammatory signalling in primary airway epithelial cells expressing Z 1-antitrypsin," Human Molecular Genetics, vol. 23, no. 4, pp. 929-941, 2014.
162. A. Di Stefano, G. Caramori, I. Gnemmi et al., "T helper type 17-related cytokine expression is increased in the bronchial mucosa of stable chronic obstructive pulmonary disease patients," Clinical & Experimental Immunology, vol. 157, no. 2, pp. 316-324, 2009.
163. C. Doe, M. Bafadhel, S. Siddiqui et al., "Expression of the T helper 17-associated cytokines IL-17A and IL-17F in asthma and COPD," Chest, vol. 138, no. 5, pp. 1140-1147, 2010.
164. E. Sapey, J. A. Stockley, H. Greenwood et al., "Behavioral and structural differences in migrating peripheral neutrophils from patients with chronic obstructive pulmonary disease," American Journal of Respiratory and Critical Care Medicine, vol. 183, no. 9, pp. 1176-1186, 2011.
165. C. S. Berenson, M. A. Garlipp, L. J. Grove, J. Maloney, and S. Sethi, "Impaired phagocytosis of nontypeable Haemophilus influenzae by human alveolar macrophages in chronic obstructive pulmonary disease," The Journal of Infectious Diseases, vol. 194, no. 10, pp. 1375-1384, 2006.
166. C. S. Berenson, C. T. Wrona, L. J. Grove et al., "Impaired alveolar macrophage response to Haemophilus antigens in chronic obstructive lung disease," American Journal of Respiratory and Critical Care Medicine, vol. 174, no. 1, pp. 31-40, 2006.
167. A. E. Taylor, T. K. Finney-Hayward, J. K. Quint et al., "Defective macrophage phagocytosis of bacteria in COPD," European Respiratory Journal, vol. 35, no. 5, pp. 1039-1047, 2010.
168. C. S. Berenson, R. L. Kruzel, E. Eberhardt et al., "Impaired innate immune alveolar macrophage response and the predilection for COPD exacerbations," Thorax, vol. 69, no. 9, pp. 811-818, 2014.
169. G. R. Van Pottelberge, K. R. Bracke, I. K. Demedts et al., "Selective accumulation of Langerhans-type dendritic cells in small airways of patients with COPD," Respiratory Research, vol. 11, no. 1, p. 35, 2010.
170. C. M. Freeman, F. J. Martinez, M. L. K. Han et al., "Lung dendritic cell expression of maturation molecules increases with worsening chronic obstructive pulmonary disease," American Journal of Respiratory and Critical Care Medicine, vol. 180, no. 12, pp. 1179-1188, 2009.
171. R. Vassallo, P. R. Walters, J. Lamont, T. J. Kottom, E. S. Yi, and A. H. Limper, "Cigarette smoke promotes dendritic cell accumulation in COPD; a lung tissue research consortium study," Respiratory Research, vol. 11, no. 1, p. 45, 2010.
172. I. K. Demedts, K. R. Bracke, G. Van Pottelberge et al., "Accumulation of dendritic cells and increased CCL20 levels in the airways of patients with chronic obstructive pulmonary disease," American Journal of Respiratory and Critical Care Medicine, vol. 175, no. 10, pp. 998-1005, 2007.
173. A. V. Rogers, E. Adelroth, K. Hattotuwa, A. Dewar, and P. K. Jeffery, "Bronchial mucosal dendritic cells in smokers and exsmokers with COPD: an electron microscopic study," Thorax, vol. 63, no. 2, pp. 108-114, 2008.
174. M. Tsoumakidou, I. Bouloukaki, H. Koutala et al., "Decreased sputum mature dendritic cells in healthy smokers and patients with chronic obstructive pulmonary disease," International Archives of Allergy and Immunology, vol. 150, no. 4, pp. 389-397, 2009.
175. S.-X. Liao, T. Ding, X.-M. Rao et al., "Cigarette smoke affects dendritic cell maturation in the small airways of patients with chronic obstructive pulmonary disease," Molecular Medicine Reports, vol. 11, no. 1, pp. 219-225, 2015.
176. J. Mjösberg and H. Spits, "Human innate lymphoid cells," Journal of Allergy and Clinical Immunology, vol. 138, no. 5, pp. 1265-1276, 2016.
177. K. C. De Grove, S. Provoost, F. M. Verhamme et al., "Characterization and quantification of innate lymphoid cell subsets in human lung," PLoS One, vol. 11, no. 1, p. e0145961, 2016.
178. J. S. Silver, J. Kearley, A. M. Copenhaver et al., "Inflammatory triggers associated with exacerbations of COPD orchestrate plasticity of group 2 innate lymphoid cells in the lungs," Nature Immunology, vol. 17, no. 6, pp. 626-635, 2016.
179. S. M. Bal, J. H. Bernink, M. Nagasawa et al., "IL-1, IL-4 and IL-12 control the fate of group 2 innate lymphoid cells in human airway inflammation in the lungs," Nature Immunology, vol. 17, no. 6, pp. 636-645, 2016.
180. A. B. Roos, C. Sandén, M. Mori, L. Bjermer, M. R. Stampfli, and J. S. Erjefält, "IL-17A is elevated in end-stage chronic obstructive pulmonary disease and contributes to cigarette smokeinduced lymphoid neogenesis," American Journal of Respiratory and Critical Care Medicine, vol. 191, no. 11, pp. 1232-1241, 2015.
181. E. Montaldo, K. Juelke, and C. Romagnani, "Group 3 innate lymphoid cells (ILC3s): origin, differentiation, and plasticity in humans and mice," European Journal of Immunology, vol. 45, no. 8, pp. 2171-2182, 2015.
182. J. C. Hogg, F. Chu, S. Utokaparch et al., "The nature of small-airway obstruction in chronic obstructive pulmonary disease," New England Journal of Medicine, vol. 350, no. 26, pp. 2645-2653, 2004.
183. B. W. A. van der Strate, D. S. Postma, C.-A. Brandsma et al., "Cigarette smoke-induced emphysema: a role for the B cell," American Journal of Respiratory and Critical Care Medicine, vol. 173, no. 7, pp. 751-758, 2006.
184. M. Mori, C. K. Andersson, K. A. Svedberg et al., "Appearance of remodelled and dendritic cell-rich alveolar-lymphoid interfaces provides a structural basis for increased alveolar antigen uptake in chronic obstructive pulmonary disease," Thorax, vol. 68, no. 6, pp. 521-531, 2013.
185. E. J. Lammertyn, E. Vandermeulen, H. Bellon et al., "Endstage cystic fibrosis lung disease is characterised by a diverse inflammatory pattern: an immunohistochemical analysis," Respiratory Research, vol. 18, no. 1, p. 10, 2017.
186. J. Frija-Masson, C. Martin, L. Regard et al., "Bacteria-driven peribronchial lymphoid neogenesis in bronchiectasis and cystic fibrosis," European Respiratory Journal, vol. 49, no. 4, p. 1601873, 2017.
187. J. A. Voynow, B. M. Fischer, B. C. Roberts, and A. D. Proia, "Basal-like cells constitute the proliferating cell population in cystic fibrosis airways," American Journal of Respiratory and Critical Care Medicine, vol. 172, no. 8, pp. 1013-8, 2005.
188. N. T. N. Trinh, O. Bardou, A. Privé et al., "Improvement of defective cystic fibrosis airway epithelial wound repair after CFTR rescue," European Respiratory Journal, vol. 40, no. 6, pp. 1390-1400, 2012.
189. C. Hubeau, M. Lorenzato, J. P. Couetil et al., "Quantitative analysis of inflammatory cells infiltrating the cystic fibrosis airway mucosa," Clinical and Experimental Immunology, vol. 124, no. 1, pp. 69-76, 2001.
190. M. W. Leigh, J. E. Kylander, J. R. Yankaskas, and R. C. Boucher, "Cell proliferation in bronchial epithelium and submucosal glands of cystic fibrosis patients," American Journal of Respiratory Cell and Molecular Biology, vol. 12, no. 6, pp. 605-612, 1995.
191. T. Piorunek, A. Marszalek, W. Biczysko, J. Gozdzik, S. Cofta, and M. Seget, "Correlation between the stage of cystic fibrosis and the level of morphological changes in adult patients," Journal of Physiology and Pharmacology, vol. 59, Supplement 6, pp. 565-572, 2008.
192. H. A. Tiddens, L. P. Koopman, R. K. Lambert et al., "Cartilaginous airway wall dimensions and airway resistance in cystic fibrosis lungs," European Respiratory Journal, vol. 15, no. 4, pp. 735-742, 2000.
193. M. Dovey, C. L. Wisseman, V. L. Roggli, G. M. Roomans, J. D. Shelburne, and A. Spock, "Ultrastructural morphology of the lung in cystic fibrosis," Journal of Submicroscopic Cytology and Pathology, vol. 21, no. 3, pp. 521-534, 1989.
194. H. Gilljam, A. M. Motakefi, B. Robertson, and B. Strandvik, "Ultrastructure of the bronchial epithelium in adult patients with cystic fibrosis," European Journal of Respiratory Diseases, vol. 71, no. 3, pp. 187-194, 1987.
195. E. H. Oppenheimer, "Similarity of the tracheobronchial mucous glands and epithelium in infants with and without cystic fibrosis," Human Pathology, vol. 12, no. 1, pp. 36-48, 1981.
196. R. W. A. Godfrey, N. J. Severs, and P. K. Jeffery, "Structural alterations of airway epithelial tight junctions in cystic fibrosis: comparison of transplant and postmortem tissue," American Journal of Respiratory Cell and Molecular Biology, vol. 9, no. 2, pp. 148-156, 1993.
197. H. A. W. M. Tiddens, S. H. Donaldson, M. Rosenfeld, and P. D. Paré, "Cystic fibrosis lung disease starts in the small airways: can we treat it more effectively," Pediatric Pulmonology, vol. 45, no. 2, pp. 107-117, 2010.
198. I. Durieu, S. Peyrol, D. Gindre, G. Bellon, D. V. Durand, and Y. Pacheco, "Subepithelial fibrosis and degradation of the bronchial extracellular matrix in cystic fibrosis," American Journal of Respiratory and Critical Care Medicine, vol. 158, no. 2, pp. 580-588, 1998.
199. S. R. Hays and J. V. Fahy, "Characterizing mucous cell remodeling in cystic fibrosis: relationship to neutrophils," American Journal of Respiratory and Critical Care Medicine, vol. 174, no. 9, pp. 1018-1024, 2006.
200. K. R. Schiller, P. J. Maniak, and S. M. O'Grady, "Cystic fibrosis transmembrane conductance regulator is involved in airway epithelial wound repair," American Journal of Physiology-Cell Physiology, vol. 299, no. 5, pp. C912-C921, 2010.
201. M. O. Wielpütz, O. Weinheimer, M. Eichinger et al., "Pulmonary emphysema in cystic fibrosis detected by densitometry on chest multidetector computed tomography," PLoS One, vol. 8, no. 8, article e73142, 2013.
202. O. M. Mets, S. M. Roothaan, I. Bronsveld et al., "Emphysema is common in lungs of cystic fibrosis lung transplantation patients: a histopathological and computed tomography study," PLoS One, vol. 10, no. 6, article e0128062, 2015.
203. I. Petrache, V. Natarajan, L. Zhen et al., "Ceramide upregulation causes pulmonary cell apoptosis and emphysema-like disease in mice," Nature Medicine, vol. 11, no. 5, pp. 491-498, 2005.
204. K. L. Kirk, "CFTR channels and wound healing. Focus on "cystic fibrosis transmembrane conductance regulator is involved in airway epithelial wound repair"," American Journal of Physiology-Cell Physiology, vol. 299, no. 5, pp. C888-C890, 2010.
205. N. T. N. Trinh, A. Privé, E. Maillé, J. Noël, and E. Brochiero, "EGF and K+ channel activity control normal and cystic fibrosis bronchial epithelia repair," American Journal of Physiology-Lung Cellular and Molecular Physiology, vol. 295, no. 5, pp. L866-L880, 2008.
206. C. M. Gosden and J. R. Gosden, "Fetal abnormalities in cystic fibrosis suggest a deficiency in proteolysis of cholecystokinin," The Lancet, vol. 324, no. 8402, pp. 541-546, 1984.
207. A. Ornoy, J. Arnon, D. Katznelson et al., "Pathological confirmation of cystic fibrosis in the fetus following prenatal diagnosis," American Journal of Medical Genetics, vol. 28, no. 4, pp. 935-947, 1987.
208. R. Hajj, P. Lesimple, B. Nawrocki-Raby, P. Birembaut, E. Puchelle, and C. Coraux, "Human airway surface epithelial regeneration is delayed and abnormal in cystic fibrosis," The Journal of Pathology, vol. 211, no. 3, pp. 340-350, 2007.
209. D. Adam, J. Roux-Delrieu, E. Luczka et al., "Cystic fibrosis airway epithelium remodelling: involvement of inflammation," The Journal of Pathology, vol. 235, no. 3, pp. 408-419, 2015.
210. I. Vachier, A. M. Vignola, G. Chiappara et al., "Inflammatory features of nasal mucosa in smokers with and without COPD," Thorax, vol. 59, no. 4, pp. 303-307, 2004.
211. P. K. Jeffery, "Comparison of the structural and inflammatory features of COPD and asthma Giles F. Filley lecture," Chest, vol. 117, no. 5, pp. 251S-260S, 2000.
212. J. F. Chmiel and M. W. Konstan, "Inflammation and antiinflammatory therapies for cystic fibrosis," Clinics in Chest Medicine, vol. 28, no. 2, pp. 331-346, 2007.
213. T. N. Hilliard, N. Regamey, J. K. Shute et al., "Airway remodelling in children with cystic fibrosis," Thorax, vol. 62, no. 12, pp. 1074-1080, 2007.
214. M. C. Bruce, L. Poncz, J. D. Klinger, R. C. Stern, Tomashefski JF Jr, and D. G. Dearborn, "Biochemical and pathologic evidence for proteolytic destruction of lung connective tissue in cystic fibrosis," American Review of Respiratory Disease, vol. 132, no. 3, pp. 529-535, 1985.
215. N. Regamey, P. K. Jeffery, E. W. F. W. Alton, A. Bush, and J. C. Davies, "Airway remodelling and its relationship to inflammation in cystic fibrosis," Thorax, vol. 66, no. 7, pp. 624-629, 2011.
216. T. Iosifidis, L. W. Garratt, D. R. Coombe, D. A. Knight, S. M. Stick, and A. Kicic, "Airway epithelial repair in health and disease: orchestrator or simply a player," Respirology, vol. 21, no. 3, pp. 438-448, 2016.
217. L. W. Garratt, E. N. Sutanto, K. M. Ling et al., "Alpha-1 antitrypsin mitigates the inhibition of airway epithelial cell repair by neutrophil elastase," American Journal of Respiratory Cell and Molecular Biology, vol. 54, no. 3, pp. 341-349, 2016.
218. A. Gaggar, A. Hector, P. E. Bratcher, M. A. Mall, M. Griese, and D. Hartl, "The role of matrix metalloproteinases in cystic fibrosis lung disease," European Respiratory Journal, vol. 38, no. 3, pp. 721-727, 2011.
219. S. D. Sagel, R. K. Kapsner, and I. Osberg, "Induced sputum matrix metalloproteinase-9 correlates with lung function and airway inflammation in children with cystic fibrosis," Pediatric Pulmonology, vol. 39, no. 3, pp. 224-232, 2005.
220. L. W. Garratt, E. N. Sutanto, K.-M. Ling et al., "Matrix metalloproteinase activation by free neutrophil elastase contributes to bronchiectasis progression in early cystic fibrosis," European Respiratory Journal, vol. 46, no. 2, pp. 384-394, 2015.
221. U. I. Sires, G. Murphy, V. M. Baragi, C. J. Fliszar, H. G. Welgus, and R. M. Senior, "Matrilysin is much more efficient than other matrix metalloproteinases in the proteolytic inactivation of 1-antitrypsin," Biochemical and Biophysical Research Communications, vol. 204, no. 2, pp. 613-620, 1994.
222. J. R. Peters-Hall, K. J. Brown, D. K. Pillai et al., "Quantitative proteomics reveals an altered cystic fibrosis in vitro bronchial epithelial secretome," American Journal of Respiratory Cell and Molecular Biology, vol. 53, no. 1, pp. 22-32, 2015.
223. Y. Itokazu, R. E. Pagano, A. S. Schroeder, S. M. O'Grady, A. H. Limper, and D. L. Marks, "Reduced GM1 ganglioside in CFTR-deficient human airway cells results in decreased 1-integrin signaling and delayed wound repair," American Journal of Physiology-Cell Physiology, vol. 306, no. 9, pp. C819-C830, 2014.
224. R. E. Sobonya and L. M. Taussig, "Quantitative aspects of lung pathology in cystic fibrosis," American Review of Respiratory Disease, vol. 134, no. 2, pp. 290-295, 1986.
225. R. Hamutcu, J. M. Rowland, M. V. Horn et al., "Clinical findings and lung pathology in children with cystic fibrosis," American Journal of Respiratory and Critical Care Medicine, vol. 165, no. 8, pp. 1172-1175, 2002.
226. M. Boon, S. E. Verleden, B. Bosch et al., "Morphometric analysis of explant lungs in cystic fibrosis," American Journal of Respiratory and Critical Care Medicine, vol. 193, no. 5, pp. 516-526, 2016.
227. M. Saetta, G. Turato, S. Baraldo et al., "Goblet cell hyperplasia and epithelial inflammation in peripheral airways of smokers with both symptoms of chronic bronchitis and chronic airflow limitation," American Journal of Respiratory and Critical Care Medicine, vol. 161, no. 3, pp. 1016-1021, 2000.
228. J. E. McDonough, R. Yuan, M. Suzuki et al., "Small-airway obstruction and emphysema in chronic obstructive pulmonary disease," New England Journal of Medicine, vol. 365, no. 17, pp. 1567-1575, 2011.
229. J. Yang, W. L. Zuo, T. Fukui et al., "Smoking-dependent distal-to-proximal repatterning of the adult human small airway epithelium," American Journal of Respiratory and Critical Care Medicine, vol. 196, no. 3, pp. 340-352, 2017.
230. W. I. de Boer, C. M. Hau, A. van Schadewijk, J. Stolk, J. H. J. M. van Krieken, and P. S. Hiemstra, "Expression of epidermal growth factors and their receptors in the bronchial epithelium of subjects with chronic obstructive pulmonary disease," American Journal of Clinical Pathology, vol. 125, no. 2, pp. 184-192, 2006.
231. J. M. Perotin, D. Adam, J. Vella-Boucaud et al., "Delay of airway epithelial wound repair in COPD is associated with airflow obstruction severity," Respiratory Research, vol. 15, no. 1, p. 151, 2014.
232. S. S. Sohal, D. Reid, A. Soltani et al., "Evaluation of epithelial mesenchymal transition in patients with chronic obstructive pulmonary disease," Respiratory Research, vol. 12, no. 1, p. 130, 2011.
233. C. Hupin, S. Gohy, C. Bouzin, M. Lecocq, M. Polette, and C. Pilette, "Features of mesenchymal transition in the airway epithelium from chronic rhinosinusitis," Allergy, vol. 69, no. 11, pp. 1540-1549, 2014.
234. H. W. Shin, K. Cho, D. W. Kim et al., "Hypoxia-inducible factor 1 mediates nasal polypogenesis by inducing epithelial-to-mesenchymal transition," American Journal of Respiratory and Critical Care Medicine, vol. 185, no. 9, pp. 944-954, 2012.
235. M. Mahmood, S. S. Sohal, S. D. Shukla et al., "Epithelial mesenchymal transition in smokers: large versus small airways and relation to airflow obstruction," International Journal of Chronic Obstructive Pulmonary Disease, vol. 10, pp. 1515-1524, 2015.
236. J. Milara, T. Peiró, A. Serrano, and J. Cortijo, "Epithelial to mesenchymal transition is increased in patients with COPD and induced by cigarette smoke," Thorax, vol. 68, no. 5, pp. 410-420, 2013.
237. S. T. Gohy, C. Hupin, C. Fregimilicka et al., "Imprinting of the COPD airway epithelium for dedifferentiation and mesenchymal transition," European Respiratory Journal, vol. 45, no. 5, pp. 1258-1272, 2015.
238. I. H. Heijink, H. G. de Bruin, R. Dennebos et al., "Cigarette smoke-induced epithelial expression of WNT-5B: implications for COPD," European Respiratory Journal, vol. 48, no. 2, pp. 504-515, 2016.
239. H. A. Baarsma, W. Skronska-Wasek, K. Mutze et al., "Noncanonical WNT-5A signaling impairs endogenous lung repair in COPD," The Journal of Experimental Medicine, vol. 214, no. 1, pp. 143-163, 2017.
240. S. T. Gohy, B. R. Detry, M. Lecocq et al., "Polymeric immunoglobulin receptor down-regulation in chronic obstructive pulmonary disease. Persistence in the cultured epithelium and role of transforming growth factor-," American Journal of Respiratory and Critical Care Medicine, vol. 190, no. 5, pp. 509-521, 2014.
241. C. Pilette, V. Godding, R. Kiss et al., "Reduced epithelial expression of secretory component in small airways correlates with airflow obstruction in chronic obstructive pulmonary disease," American Journal of Respiratory and Critical Care Medicine, vol. 163, no. 1, pp. 185-194, 2001.
242. B. W. Richmond, R. M. Brucker, W. Han et al., "Airway bacteria drive a progressive COPD-like phenotype in mice with polymeric immunoglobulin receptor deficiency," Nature Communications, vol. 7, article 11240, 2016.
243. S. S. Sohal, A. Soltani, D. Reid et al., "A randomized controlled trial of inhaled corticosteroids (ICS) on markers of epithelial-mesenchymal transition (EMT) in large airway samples in COPD: an exploratory proof of concept study," International Journal of Chronic Obstructive Pulmonary Disease, vol. 9, pp. 533-542, 2014.
244. M. Podowski, C. Calvi, S. Metzger et al., "Angiotensin receptor blockade attenuates cigarette smoke-induced lung injury and rescues lung architecture in mice," Journal of Clinical Investigation, vol. 122, no. 1, pp. 229-240, 2012.