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Volumn 34, Issue , 2017, Pages 132-139

The therapeutic potential of CFTR modulators for COPD and other airway diseases

Author keywords

[No Author keywords available]

Indexed keywords

BRONCHODILATING AGENT; CORTICOSTEROID; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; IVACAFTOR; PHOSPHODIESTERASE IV INHIBITOR; ROFLUMILAST; STEROID;

EID: 85033367251     PISSN: 14714892     EISSN: 14714973     Source Type: Journal    
DOI: 10.1016/j.coph.2017.09.013     Document Type: Review
Times cited : (49)

References (73)
  • 2
    • 84873353231 scopus 로고    scopus 로고
    • Chronic bronchitis and chronic obstructive pulmonary disease
    • Kim, V., Criner, G.J., Chronic bronchitis and chronic obstructive pulmonary disease. Am J Respir Crit Care Med 187 (2013), 228–237.
    • (2013) Am J Respir Crit Care Med , vol.187 , pp. 228-237
    • Kim, V.1    Criner, G.J.2
  • 3
    • 84887976747 scopus 로고    scopus 로고
    • Severe chronic bronchitis in advanced emphysema increases mortality and hospitalizations
    • Kim, V., Sternberg, A.L., Washko, G., Make, B.J., Han, M.K., Martinez, F., Criner, G.J., National Emphysema Treatment Trial Research G. Severe chronic bronchitis in advanced emphysema increases mortality and hospitalizations. COPD 10 (2013), 667–678.
    • (2013) COPD , vol.10 , pp. 667-678
    • Kim, V.1    Sternberg, A.L.2    Washko, G.3    Make, B.J.4    Han, M.K.5    Martinez, F.6    Criner, G.J.7
  • 4
    • 84959476027 scopus 로고    scopus 로고
    • Acquired cystic fibrosis transmembrane conductance regulator dysfunction in chronic bronchitis and other diseases of mucus clearance
    • Review that describes the mechanistic basis for acquired CFTR dysfunction in chronic bronchitis.
    • Raju, S.V., Solomon, G.M., Dransfield, M.T., Rowe, S.M., Acquired cystic fibrosis transmembrane conductance regulator dysfunction in chronic bronchitis and other diseases of mucus clearance. Clin Chest Med 37 (2016), 147–158 Review that describes the mechanistic basis for acquired CFTR dysfunction in chronic bronchitis.
    • (2016) Clin Chest Med , vol.37 , pp. 147-158
    • Raju, S.V.1    Solomon, G.M.2    Dransfield, M.T.3    Rowe, S.M.4
  • 6
    • 33646540731 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers
    • First evidence that CFTR expression was decreased by exposure to cigarette smoke in vitro and in vivo. They showed that acquired CFTR deficiency occurs in the nasal respiratory epithelium of cigarette smokers and suggested that CFTR deficiency contributed to cigarette-induced diseases such as chronic bronchitis.
    • Cantin, A.M., Hanrahan, J.W., Bilodeau, G., Ellis, L., Dupuis, A., Liao, J., Zielenski, J., Durie, P., Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers. Am J Respir Crit Care Med 173 (2006), 1139–1144 First evidence that CFTR expression was decreased by exposure to cigarette smoke in vitro and in vivo. They showed that acquired CFTR deficiency occurs in the nasal respiratory epithelium of cigarette smokers and suggested that CFTR deficiency contributed to cigarette-induced diseases such as chronic bronchitis.
    • (2006) Am J Respir Crit Care Med , vol.173 , pp. 1139-1144
    • Cantin, A.M.1    Hanrahan, J.W.2    Bilodeau, G.3    Ellis, L.4    Dupuis, A.5    Liao, J.6    Zielenski, J.7    Durie, P.8
  • 7
    • 84881494429 scopus 로고    scopus 로고
    • Acquired cystic fibrosis transmembrane conductance regulator dysfunction in the lower airways in COPD
    • This important study compared lower airway CFTR activity in current and former smokers with COPD, current smokers without COPD, and nonsmokers and found that smokers with and without COPD had reduced lower airway activity compared with nonsmokers and suggested that CFTR dysfunction contributed to COPD pathogenesis.
    • Dransfield, M.T., Wilhelm, A.M., Flanagan, B., Courville, C., Tidwell, S.L., Raju, S.V., Gaggar, A., Steele, C., Tang, L.P., Liu, B., Rowe, S.M., Acquired cystic fibrosis transmembrane conductance regulator dysfunction in the lower airways in COPD. Chest 144 (2013), 498–506 This important study compared lower airway CFTR activity in current and former smokers with COPD, current smokers without COPD, and nonsmokers and found that smokers with and without COPD had reduced lower airway activity compared with nonsmokers and suggested that CFTR dysfunction contributed to COPD pathogenesis.
    • (2013) Chest , vol.144 , pp. 498-506
    • Dransfield, M.T.1    Wilhelm, A.M.2    Flanagan, B.3    Courville, C.4    Tidwell, S.L.5    Raju, S.V.6    Gaggar, A.7    Steele, C.8    Tang, L.P.9    Liu, B.10    Rowe, S.M.11
  • 8
    • 84908133390 scopus 로고    scopus 로고
    • Correlation of apical fluid-regulating channel proteins with lung function in human copd lungs
    • Zhao, R., Liang, X., Zhao, M., Liu, S.L., Huang, Y., Idell, S., Li, X., Ji, H.L., Correlation of apical fluid-regulating channel proteins with lung function in human copd lungs. PLOS ONE, 9, 2014, e109725.
    • (2014) PLOS ONE , vol.9 , pp. e109725
    • Zhao, R.1    Liang, X.2    Zhao, M.3    Liu, S.L.4    Huang, Y.5    Idell, S.6    Li, X.7    Ji, H.L.8
  • 9
    • 84899486875 scopus 로고    scopus 로고
    • Acquired defects in CFTR-dependent beta-adrenergic sweat secretion in chronic obstructive pulmonary disease
    • Courville, C.A., Tidwell, S., Liu, B., Accurso, F.J., Dransfield, M.T., Rowe, S.M., Acquired defects in CFTR-dependent beta-adrenergic sweat secretion in chronic obstructive pulmonary disease. Respir Res, 15, 2014, 25.
    • (2014) Respir Res , vol.15 , pp. 25
    • Courville, C.A.1    Tidwell, S.2    Liu, B.3    Accurso, F.J.4    Dransfield, M.T.5    Rowe, S.M.6
  • 10
    • 84951975635 scopus 로고    scopus 로고
    • Recovery of acquired cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction following smoking cessation
    • Courville, C.A., Raju, S.V., Liu, B., Accurso, F.J., Dransfield, M.T., Rowe, S.M., Recovery of acquired cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction following smoking cessation. Am J Respir Crit Care Med 192:12 (2015), 1521–1524.
    • (2015) Am J Respir Crit Care Med , vol.192 , Issue.12 , pp. 1521-1524
    • Courville, C.A.1    Raju, S.V.2    Liu, B.3    Accurso, F.J.4    Dransfield, M.T.5    Rowe, S.M.6
  • 12
    • 84890050882 scopus 로고    scopus 로고
    • Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function
    • This significant paper illustrated that cigarette smoke affected other organ systems such as intestinal epithelium and showed that acrolein, a component of cigarette smoke, was higher in smokers and inhibited CFTR channel gating, and that a scavenger of acrolein, N-acetylcysteine, could reverse these effects.
    • Raju, S.V., Jackson, P.L., Courville, C.A., McNicholas, C.M., Sloane, P.A., Sabbatini, G., Tidwell, S., Tang, L.P., Liu, B., Fortenberry, J.A., Jones, C.W., et al. Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function. Am J Respir Crit Care Med 188 (2013), 1321–1330 This significant paper illustrated that cigarette smoke affected other organ systems such as intestinal epithelium and showed that acrolein, a component of cigarette smoke, was higher in smokers and inhibited CFTR channel gating, and that a scavenger of acrolein, N-acetylcysteine, could reverse these effects.
    • (2013) Am J Respir Crit Care Med , vol.188 , pp. 1321-1330
    • Raju, S.V.1    Jackson, P.L.2    Courville, C.A.3    McNicholas, C.M.4    Sloane, P.A.5    Sabbatini, G.6    Tidwell, S.7    Tang, L.P.8    Liu, B.9    Fortenberry, J.A.10    Jones, C.W.11
  • 13
    • 84978882359 scopus 로고    scopus 로고
    • Nicotine exposure induces bronchial epithelial cell apoptosis and senescence via ros mediated autophagy-impairment
    • Bodas, M., Van Westphal, C., Carpenter-Thompson, R., D, K.M., Vij, N., Nicotine exposure induces bronchial epithelial cell apoptosis and senescence via ros mediated autophagy-impairment. Free Radic Biol Med 97 (2016), 441–453.
    • (2016) Free Radic Biol Med , vol.97 , pp. 441-453
    • Bodas, M.1    Van Westphal, C.2    Carpenter-Thompson, R.3    D, K.M.4    Vij, N.5
  • 14
    • 84896273138 scopus 로고    scopus 로고
    • Cigarette smoke-induced Ca2+ release leads to cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction
    • Rasmussen, J.E., Sheridan, J.T., Polk, W., Davies, C.M., Tarran, R., Cigarette smoke-induced Ca2+ release leads to cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction. J Biol Chem 289 (2014), 7671–7681.
    • (2014) J Biol Chem , vol.289 , pp. 7671-7681
    • Rasmussen, J.E.1    Sheridan, J.T.2    Polk, W.3    Davies, C.M.4    Tarran, R.5
  • 15
    • 84951064927 scopus 로고    scopus 로고
    • Will chronic e-cigarette use cause lung disease?
    • Rowell, T.R., Tarran, R., Will chronic e-cigarette use cause lung disease?. Am J Physiol Lung Cell Mol Physiol 309 (2015), L1398–L1409.
    • (2015) Am J Physiol Lung Cell Mol Physiol , vol.309 , pp. L1398-L1409
    • Rowell, T.R.1    Tarran, R.2
  • 16
    • 84856557879 scopus 로고    scopus 로고
    • Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration
    • In this mechanistic study, the authors demonstrate that cigarette exposure directly leads to loss of CFTR from the cell surface and results in airway surface dehydration, leading to mucus stasis, and suggested that stategies for rehydrating the surface could provide a therapy for chronic bronchitis.
    • Clunes, L.A., Davies, C.M., Coakley, R.D., Aleksandrov, A.A., Henderson, A.G., Zeman, K.L., Worthington, E.N., Gentzsch, M., Kreda, S.M., Cholon, D., Bennett, W.D., et al. Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration. FASEB J 26 (2012), 533–545 In this mechanistic study, the authors demonstrate that cigarette exposure directly leads to loss of CFTR from the cell surface and results in airway surface dehydration, leading to mucus stasis, and suggested that stategies for rehydrating the surface could provide a therapy for chronic bronchitis.
    • (2012) FASEB J , vol.26 , pp. 533-545
    • Clunes, L.A.1    Davies, C.M.2    Coakley, R.D.3    Aleksandrov, A.A.4    Henderson, A.G.5    Zeman, K.L.6    Worthington, E.N.7    Gentzsch, M.8    Kreda, S.M.9    Cholon, D.10    Bennett, W.D.11
  • 18
    • 84872502797 scopus 로고    scopus 로고
    • Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo
    • This manuscipt provides a novel a link between neutrophil elastase-calpain activation and CFTR loss of function in bacterial lung infections relevant to CF. They demonstrate that neutrophil elastase degrades CFTR through activation of intracellular calpains.
    • Le Gars, M., Descamps, D., Roussel, D., Saussereau, E., Guillot, L., Ruffin, M., Tabary, O., Hong, S.S., Boulanger, P., Paulais, M., Malleret, L., et al. Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo. Am J Respir Crit Care Med 187 (2013), 170–179 This manuscipt provides a novel a link between neutrophil elastase-calpain activation and CFTR loss of function in bacterial lung infections relevant to CF. They demonstrate that neutrophil elastase degrades CFTR through activation of intracellular calpains.
    • (2013) Am J Respir Crit Care Med , vol.187 , pp. 170-179
    • Le Gars, M.1    Descamps, D.2    Roussel, D.3    Saussereau, E.4    Guillot, L.5    Ruffin, M.6    Tabary, O.7    Hong, S.S.8    Boulanger, P.9    Paulais, M.10    Malleret, L.11
  • 20
    • 0029611346 scopus 로고
    • Analysis of respiratory mucus glycoproteins in asthma: a detailed study from a patient who died in status asthmaticus
    • Sheehan, J.K., Richardson, P.S., Fung, D.C., Howard, M., Thornton, D.J., Analysis of respiratory mucus glycoproteins in asthma: a detailed study from a patient who died in status asthmaticus. Am J Respir Cell Mol Biol 13 (1995), 748–756.
    • (1995) Am J Respir Cell Mol Biol , vol.13 , pp. 748-756
    • Sheehan, J.K.1    Richardson, P.S.2    Fung, D.C.3    Howard, M.4    Thornton, D.J.5
  • 21
    • 78649735324 scopus 로고    scopus 로고
    • Airway mucus function and dysfunction
    • Fahy, J.V., Dickey, B.F., Airway mucus function and dysfunction. N Engl J Med 363 (2010), 2233–2247.
    • (2010) N Engl J Med , vol.363 , pp. 2233-2247
    • Fahy, J.V.1    Dickey, B.F.2
  • 24
    • 34248144056 scopus 로고    scopus 로고
    • The impact of bronchiectasis in clinical presentation of asthma
    • Oguzulgen, I.K., Kervan, F., Ozis, T., Turktas, H., The impact of bronchiectasis in clinical presentation of asthma. South Med J 100 (2007), 468–471.
    • (2007) South Med J , vol.100 , pp. 468-471
    • Oguzulgen, I.K.1    Kervan, F.2    Ozis, T.3    Turktas, H.4
  • 25
    • 84860536505 scopus 로고    scopus 로고
    • The role of CFTR mutations in asthma
    • Sandford, A., The role of CFTR mutations in asthma. Can Respir J 19 (2012), 44–45.
    • (2012) Can Respir J , vol.19 , pp. 44-45
    • Sandford, A.1
  • 26
    • 84861922716 scopus 로고    scopus 로고
    • Association of CFTR gene mutation with bronchial asthma
    • Maurya, N., Awasthi, S., Dixit, P., Association of CFTR gene mutation with bronchial asthma. Indian J Med Res 135 (2012), 469–478.
    • (2012) Indian J Med Res , vol.135 , pp. 469-478
    • Maurya, N.1    Awasthi, S.2    Dixit, P.3
  • 27
    • 0035722764 scopus 로고    scopus 로고
    • ‘CFTR-opathies’: disease phenotypes associated with cystic fibrosis transmembrane regulator gene mutations
    • Noone, P.G., Knowles, M.R., ‘CFTR-opathies’: disease phenotypes associated with cystic fibrosis transmembrane regulator gene mutations. Respir Res 2 (2001), 328–332.
    • (2001) Respir Res , vol.2 , pp. 328-332
    • Noone, P.G.1    Knowles, M.R.2
  • 28
    • 84860506911 scopus 로고    scopus 로고
    • Cystic fibrosis transmembrane conductance regulator gene abnormalities in patients with asthma and recurrent neutrophilic bronchitis
    • Goodwin, J., Spitale, N., Yaghi, A., Dolovich, M., Nair, P., Cystic fibrosis transmembrane conductance regulator gene abnormalities in patients with asthma and recurrent neutrophilic bronchitis. Can Respir J 19 (2012), 46–48.
    • (2012) Can Respir J , vol.19 , pp. 46-48
    • Goodwin, J.1    Spitale, N.2    Yaghi, A.3    Dolovich, M.4    Nair, P.5
  • 29
    • 34347262057 scopus 로고    scopus 로고
    • Interleukin-13 interferes with CFTR and AQP5 expression and localization during human airway epithelial cell differentiation
    • Skowron-zwarg, M., Boland, S., Caruso, N., Coraux, C., Marano, F., Tournier, F., Interleukin-13 interferes with CFTR and AQP5 expression and localization during human airway epithelial cell differentiation. Exp Cell Res 313 (2007), 2695–2702.
    • (2007) Exp Cell Res , vol.313 , pp. 2695-2702
    • Skowron-zwarg, M.1    Boland, S.2    Caruso, N.3    Coraux, C.4    Marano, F.5    Tournier, F.6
  • 30
    • 0036081034 scopus 로고    scopus 로고
    • Interleukin-13 induces a hypersecretory ion transport phenotype in human bronchial epithelial cells
    • Danahay, H., Atherton, H., Jones, G., Bridges, R.J., Poll, C.T., Interleukin-13 induces a hypersecretory ion transport phenotype in human bronchial epithelial cells. Am J Physiol Lung Cell Mol Physiol 282 (2002), L226–L236.
    • (2002) Am J Physiol Lung Cell Mol Physiol , vol.282 , pp. L226-L236
    • Danahay, H.1    Atherton, H.2    Jones, G.3    Bridges, R.J.4    Poll, C.T.5
  • 32
    • 78649733432 scopus 로고    scopus 로고
    • Allergic airway inflammation induces a pro-secretory epithelial ion transport phenotype in mice
    • Anagnostopoulou, P., Dai, L., Schatterny, J., Hirtz, S., Duerr, J., Mall, M.A., Allergic airway inflammation induces a pro-secretory epithelial ion transport phenotype in mice. Eur Respir J 36 (2010), 1436–1447.
    • (2010) Eur Respir J , vol.36 , pp. 1436-1447
    • Anagnostopoulou, P.1    Dai, L.2    Schatterny, J.3    Hirtz, S.4    Duerr, J.5    Mall, M.A.6
  • 33
    • 84990070540 scopus 로고    scopus 로고
    • Non-allergic asthma as a CFTR-related disorder
    • Schulz, A., Tummler, B., Non-allergic asthma as a CFTR-related disorder. J Cyst Fibros 15 (2016), 641–644.
    • (2016) J Cyst Fibros , vol.15 , pp. 641-644
    • Schulz, A.1    Tummler, B.2
  • 34
    • 84873184252 scopus 로고    scopus 로고
    • Antioxidants and mucolytics in copd management: when (if ever) and in whom?
    • Hillas, G., Nikolakopoulou, S., Hussain, S., Vassilakopoulos, T., Antioxidants and mucolytics in copd management: when (if ever) and in whom?. Curr Drug Targets 14 (2013), 225–234.
    • (2013) Curr Drug Targets , vol.14 , pp. 225-234
    • Hillas, G.1    Nikolakopoulou, S.2    Hussain, S.3    Vassilakopoulos, T.4
  • 35
    • 34247478107 scopus 로고    scopus 로고
    • Pathogenesis of bronchiectasis
    • Morrissey, B.M., Pathogenesis of bronchiectasis. Clin Chest Med 28 (2007), 289–296.
    • (2007) Clin Chest Med , vol.28 , pp. 289-296
    • Morrissey, B.M.1
  • 36
    • 84989261668 scopus 로고    scopus 로고
    • Neutrophil fates in bronchiectasis and alpha-1 antitrypsin deficiency
    • Russell, D.W., Gaggar, A., Solomon, G.M., Neutrophil fates in bronchiectasis and alpha-1 antitrypsin deficiency. Ann Am Thorac Soc 13:Suppl. 2 (2016), S123–S129.
    • (2016) Ann Am Thorac Soc , vol.13 , pp. S123-S129
    • Russell, D.W.1    Gaggar, A.2    Solomon, G.M.3
  • 40
    • 0036261061 scopus 로고    scopus 로고
    • Copd: Epidemiology, prevalence, morbidity and mortality, and disease heterogeneity
    • Mannino, D.M., Copd: Epidemiology, prevalence, morbidity and mortality, and disease heterogeneity. Chest 121:Suppl. (2002), 121S–126S.
    • (2002) Chest , vol.121 , pp. 121S-126S
    • Mannino, D.M.1
  • 41
    • 57049135358 scopus 로고    scopus 로고
    • Inhaled corticosteroids in patients with stable chronic obstructive pulmonary disease: a systematic review and meta-analysis
    • Drummond, M.B., Dasenbrook, E.C., Pitz, M.W., Murphy, D.J., Fan, E., Inhaled corticosteroids in patients with stable chronic obstructive pulmonary disease: a systematic review and meta-analysis. JAMA 300 (2008), 2407–2416.
    • (2008) JAMA , vol.300 , pp. 2407-2416
    • Drummond, M.B.1    Dasenbrook, E.C.2    Pitz, M.W.3    Murphy, D.J.4    Fan, E.5
  • 45
    • 85033362867 scopus 로고    scopus 로고
    • Risk of pneumonia associated with long-term use of inhaled corticosteroids in chronic obstructive pulmonary disease: a critical review and update
    • Singh, S., Loke, Y.K., Risk of pneumonia associated with long-term use of inhaled corticosteroids in chronic obstructive pulmonary disease: a critical review and update. Curr Opin Pulm Med, 2009.
    • (2009) Curr Opin Pulm Med
    • Singh, S.1    Loke, Y.K.2
  • 47
    • 44849100529 scopus 로고    scopus 로고
    • Effect of carbocisteine on acute exacerbation of chronic obstructive pulmonary disease (peace study): a randomised placebo-controlled study
    • Zheng, J.P., Kang, J., Huang, S.G., Chen, P., Yao, W.Z., Yang, L., Bai, C.X., Wang, C.Z., Wang, C., Chen, B.Y., Shi, Y., et al. Effect of carbocisteine on acute exacerbation of chronic obstructive pulmonary disease (peace study): a randomised placebo-controlled study. Lancet 371 (2008), 2013–2018.
    • (2008) Lancet , vol.371 , pp. 2013-2018
    • Zheng, J.P.1    Kang, J.2    Huang, S.G.3    Chen, P.4    Yao, W.Z.5    Yang, L.6    Bai, C.X.7    Wang, C.Z.8    Wang, C.9    Chen, B.Y.10    Shi, Y.11
  • 48
    • 33750138265 scopus 로고    scopus 로고
    • Mucolytic agents for chronic bronchitis or chronic obstructive pulmonary disease
    • Poole, P.J., Black, P.N., Mucolytic agents for chronic bronchitis or chronic obstructive pulmonary disease. Cochrane Database Syst Rev, 3, 2006, CD001287.
    • (2006) Cochrane Database Syst Rev , vol.3 , pp. CD001287
    • Poole, P.J.1    Black, P.N.2
  • 49
    • 0034640865 scopus 로고    scopus 로고
    • Obstructive lung disease and low lung function in adults in the united states: data from the national health and nutrition examination survey, 1988–1994
    • Mannino, D.M., Gagnon, R.C., Petty, T.L., Lydick, E., Obstructive lung disease and low lung function in adults in the united states: data from the national health and nutrition examination survey, 1988–1994. Arch Intern Med 160 (2000), 1683–1689.
    • (2000) Arch Intern Med , vol.160 , pp. 1683-1689
    • Mannino, D.M.1    Gagnon, R.C.2    Petty, T.L.3    Lydick, E.4
  • 50
    • 35448992094 scopus 로고    scopus 로고
    • Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbation: a pilot study
    • Dovey, M., Aitken, M.L., Emerson, J., McNamara, S., Waltz, D.A., Gibson, R.L., Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbation: a pilot study. Chest 132 (2007), 1212–1218.
    • (2007) Chest , vol.132 , pp. 1212-1218
    • Dovey, M.1    Aitken, M.L.2    Emerson, J.3    McNamara, S.4    Waltz, D.A.5    Gibson, R.L.6
  • 51
    • 84863798119 scopus 로고    scopus 로고
    • A pilot study of the impact of high-frequency chest wall oscillation in chronic obstructive pulmonary disease patients with mucus hypersecretion
    • Chakravorty, I., Chahal, K., Austin, G., A pilot study of the impact of high-frequency chest wall oscillation in chronic obstructive pulmonary disease patients with mucus hypersecretion. Int J Chron Obstruct Pulmon Dis 6 (2011), 693–699.
    • (2011) Int J Chron Obstruct Pulmon Dis , vol.6 , pp. 693-699
    • Chakravorty, I.1    Chahal, K.2    Austin, G.3
  • 54
    • 84959055667 scopus 로고    scopus 로고
    • treatment of cystic fibrosis with CFTR modulators
    • Tummler, B., treatment of cystic fibrosis with CFTR modulators. Pneumologie 70 (2016), 301–313.
    • (2016) Pneumologie , vol.70 , pp. 301-313
    • Tummler, B.1
  • 55
    • 84941166975 scopus 로고    scopus 로고
    • Breakthrough therapies: cystic fibrosis (CF) potentiators and correctors
    • Solomon, G.M., Marshall, S.G., Ramsey, B.W., Rowe, S.M., Breakthrough therapies: cystic fibrosis (CF) potentiators and correctors. Pediatr Pulmonol 50:Suppl. 40 (2015), S3–S13.
    • (2015) Pediatr Pulmonol , vol.50 , pp. S3-S13
    • Solomon, G.M.1    Marshall, S.G.2    Ramsey, B.W.3    Rowe, S.M.4
  • 56
    • 85008428244 scopus 로고    scopus 로고
    • The cystic fibrosis transmembrane conductance regulator potentiator ivacaftor augments mucociliary clearance abrogating cystic fibrosis transmembrane conductance regulator inhibition by cigarette smoke
    • In this study, the investigators show that cigarette smoke extract reduces CFTR-dependent current in human bronchial epithelial cells and human bronchi within minutes of exposure and the mechamism involved was reduction of CFTR gating. They showed that cigarette smoke extract treatment of purified CFTR resulted in acrolein modifications in amino acid residues likely to disrupt CFTR gating. Importantly, they also demonstrated that cigarette smoke extract reduced airway surface liquid depth and ciliary beat frequency and these effects could be blocked with ivacaftor coadministration in vitro.
    • Raju, S.V., Lin, V.Y., Liu, L., McNicholas, C.M., Karki, S., Sloane, P.A., Tang, L., Jackson, P.L., Wang, W., Wilson, L., Macon, K.J., et al. The cystic fibrosis transmembrane conductance regulator potentiator ivacaftor augments mucociliary clearance abrogating cystic fibrosis transmembrane conductance regulator inhibition by cigarette smoke. Am J Respir Cell Mol Biol 56 (2017), 99–108 In this study, the investigators show that cigarette smoke extract reduces CFTR-dependent current in human bronchial epithelial cells and human bronchi within minutes of exposure and the mechamism involved was reduction of CFTR gating. They showed that cigarette smoke extract treatment of purified CFTR resulted in acrolein modifications in amino acid residues likely to disrupt CFTR gating. Importantly, they also demonstrated that cigarette smoke extract reduced airway surface liquid depth and ciliary beat frequency and these effects could be blocked with ivacaftor coadministration in vitro.
    • (2017) Am J Respir Cell Mol Biol , vol.56 , pp. 99-108
    • Raju, S.V.1    Lin, V.Y.2    Liu, L.3    McNicholas, C.M.4    Karki, S.5    Sloane, P.A.6    Tang, L.7    Jackson, P.L.8    Wang, W.9    Wilson, L.10    Macon, K.J.11
  • 57
    • 84904418904 scopus 로고    scopus 로고
    • Clinical mechanism of the CFTR potentiator ivacaftor in g551d-mediated cystic fibrosis
    • This clinical study evaluated invacaftor in a postapproval setting to determine mechanism of action and the response of clinically relevant markers. The results indicated that the treated patients improved in lung function, body mass index, their hospitalization rate, and in Pseudomonas aeruginasa burden, all of which illustrated the therapeutic benefit of ivacaftor.
    • Rowe, S.M., Heltshe, S.L., Gonska, T., Donaldson, S.H., Borowitz, D., Gelfond, D., Sagel, S.D., Khan, U., Mayer-Hamblett, N., Van Dalfsen, J.M., Joseloff, E., et al. Clinical mechanism of the CFTR potentiator ivacaftor in g551d-mediated cystic fibrosis. Am J Respir Crit Care Med, 2014 This clinical study evaluated invacaftor in a postapproval setting to determine mechanism of action and the response of clinically relevant markers. The results indicated that the treated patients improved in lung function, body mass index, their hospitalization rate, and in Pseudomonas aeruginasa burden, all of which illustrated the therapeutic benefit of ivacaftor.
    • (2014) Am J Respir Crit Care Med
    • Rowe, S.M.1    Heltshe, S.L.2    Gonska, T.3    Donaldson, S.H.4    Borowitz, D.5    Gelfond, D.6    Sagel, S.D.7    Khan, U.8    Mayer-Hamblett, N.9    Van Dalfsen, J.M.10    Joseloff, E.11
  • 58
    • 73249114731 scopus 로고    scopus 로고
    • Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, Vx-770
    • Original mauscript describing the mechanistic basis of CFTR potentiation by Vx-770 (ivacaftor).
    • Van Goor, F., Hadida, S., Grootenhuis, P.D., Burton, B., Cao, D., Neuberger, T., Turnbull, A., Singh, A., Joubran, J., Hazlewood, A., Zhou, J., et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, Vx-770. Proc Natl Acad Sci U S A 106 (2009), 18825–18830 Original mauscript describing the mechanistic basis of CFTR potentiation by Vx-770 (ivacaftor).
    • (2009) Proc Natl Acad Sci U S A , vol.106 , pp. 18825-18830
    • Van Goor, F.1    Hadida, S.2    Grootenhuis, P.D.3    Burton, B.4    Cao, D.5    Neuberger, T.6    Turnbull, A.7    Singh, A.8    Joubran, J.9    Hazlewood, A.10    Zhou, J.11
  • 59
    • 84890435909 scopus 로고    scopus 로고
    • Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function
    • In this important study, the investigators show that ivacaftor, a drug effective in patients 6 years and older who have the G551D-CFTR gating mutation, is also effective other mutant forms of CFTR. This study emphasized that this drug could provide clinical benefit for a much larger spectrum of CF patients with mild defects in either CFTR processing or in channel conductance.
    • Van Goor, F., Yu, H., Burton, B., Hoffman, B.J., Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function. J Cyst Fibrosis 13 (2014), 29–36 In this important study, the investigators show that ivacaftor, a drug effective in patients 6 years and older who have the G551D-CFTR gating mutation, is also effective other mutant forms of CFTR. This study emphasized that this drug could provide clinical benefit for a much larger spectrum of CF patients with mild defects in either CFTR processing or in channel conductance.
    • (2014) J Cyst Fibrosis , vol.13 , pp. 29-36
    • Van Goor, F.1    Yu, H.2    Burton, B.3    Hoffman, B.J.4
  • 60
    • 84884773595 scopus 로고    scopus 로고
    • Vx-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1
    • Ren, H.Y., Grove, D.E., De La Rosa, O., Houck, S.A., Sopha, P., Van Goor, F., Hoffman, B.J., Cyr, D.M., Vx-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1. Mol Biol Cell 24 (2013), 3016–3024.
    • (2013) Mol Biol Cell , vol.24 , pp. 3016-3024
    • Ren, H.Y.1    Grove, D.E.2    De La Rosa, O.3    Houck, S.A.4    Sopha, P.5    Van Goor, F.6    Hoffman, B.J.7    Cyr, D.M.8
  • 63
    • 11144240244 scopus 로고    scopus 로고
    • Regulation of airway surface liquid volume and mucus transport by active ion transport
    • Tarran, R., Regulation of airway surface liquid volume and mucus transport by active ion transport. Proc Am Thorac Soc 1 (2004), 42–46.
    • (2004) Proc Am Thorac Soc , vol.1 , pp. 42-46
    • Tarran, R.1
  • 64
    • 11044226335 scopus 로고    scopus 로고
    • Measurements of airway surface liquid height and mucus transport by fluorescence microscopy, and of ion composition by X-ray microanalysis
    • Roomans, G.M., Kozlova, I., Nilsson, H., Vanthanouvong, V., Button, B., Tarran, R., Measurements of airway surface liquid height and mucus transport by fluorescence microscopy, and of ion composition by X-ray microanalysis. J Cyst Fibrosis 3:Suppl. 2 (2004), 135–139.
    • (2004) J Cyst Fibrosis , vol.3 , pp. 135-139
    • Roomans, G.M.1    Kozlova, I.2    Nilsson, H.3    Vanthanouvong, V.4    Button, B.5    Tarran, R.6
  • 65
    • 0032530370 scopus 로고    scopus 로고
    • Coordinated clearance of periciliary liquid and mucus from airway surfaces
    • Matsui, H., Randell, S.H., Peretti, S.W., Davis, C.W., Boucher, R.C., Coordinated clearance of periciliary liquid and mucus from airway surfaces. J Clin Investig 102 (1998), 1125–1131.
    • (1998) J Clin Investig , vol.102 , pp. 1125-1131
    • Matsui, H.1    Randell, S.H.2    Peretti, S.W.3    Davis, C.W.4    Boucher, R.C.5
  • 66
    • 34548215173 scopus 로고    scopus 로고
    • Mucociliary clearance as an outcome measure for cystic fibrosis clinical research
    • Donaldson, S.H., Corcoran, T.E., Laube, B.L., Bennett, W.D., Mucociliary clearance as an outcome measure for cystic fibrosis clinical research. Proc Am Thorac Soc 4 (2007), 399–405.
    • (2007) Proc Am Thorac Soc , vol.4 , pp. 399-405
    • Donaldson, S.H.1    Corcoran, T.E.2    Laube, B.L.3    Bennett, W.D.4
  • 68
    • 84966861817 scopus 로고    scopus 로고
    • Pilot evaluation of ivacaftor for chronic bronchitis
    • First Clinical description of Ivacaftor administration in COPD patients with the chronic bronchitis phenotype. While this study was primarily intended to demonstrate safety, early efficacy in patients with reduced CFTR function was observed in markers of CFTR function and symptoms scores.
    • Solomon, G.M., Hathorne, H., Liu, B., Raju, S.V., Reeves, G., Acosta, E.P., Dransfield, M.T., Rowe, S.M., Pilot evaluation of ivacaftor for chronic bronchitis. Lancet Respir Med 4 (2016), e32–e33 First Clinical description of Ivacaftor administration in COPD patients with the chronic bronchitis phenotype. While this study was primarily intended to demonstrate safety, early efficacy in patients with reduced CFTR function was observed in markers of CFTR function and symptoms scores.
    • (2016) Lancet Respir Med , vol.4 , pp. e32-e33
    • Solomon, G.M.1    Hathorne, H.2    Liu, B.3    Raju, S.V.4    Reeves, G.5    Acosta, E.P.6    Dransfield, M.T.7    Rowe, S.M.8
  • 69
    • 84989246358 scopus 로고    scopus 로고
    • Therapeutic approaches to acquired cystic fibrosis transmembrane conductance regulator dysfunction in chronic bronchitis
    • Solomon, G.M., Raju, S.V., Dransfield, M.T., Rowe, S.M., Therapeutic approaches to acquired cystic fibrosis transmembrane conductance regulator dysfunction in chronic bronchitis. Ann Am Thorac Soc 13:Suppl. 2 (2016), S169–S176.
    • (2016) Ann Am Thorac Soc , vol.13 , pp. S169-S176
    • Solomon, G.M.1    Raju, S.V.2    Dransfield, M.T.3    Rowe, S.M.4
  • 70
    • 79251588730 scopus 로고    scopus 로고
    • Reduction of exacerbations by the pde4 inhibitor roflumilast — the importance of defining different subsets of patients with copd
    • Rennard, S.I., Calverley, P.M., Goehring, U.M., Bredenbroker, D., Martinez, F.J., Reduction of exacerbations by the pde4 inhibitor roflumilast — the importance of defining different subsets of patients with copd. Respir Res, 12, 2011.
    • (2011) Respir Res , vol.12
    • Rennard, S.I.1    Calverley, P.M.2    Goehring, U.M.3    Bredenbroker, D.4    Martinez, F.J.5
  • 71
    • 69149098943 scopus 로고    scopus 로고
    • Roflumilast in moderate-to-severe chronic obstructive pulmonary disease treated with longacting bronchodilators: two randomised clinical trials
    • Fabbri, L.M., Calverley, P.M., Izquierdo-Alonso, J.L., Bundschuh, D.S., Brose, M., Martinez, F.J., Rabe, K.F., M. groups Ms. Roflumilast in moderate-to-severe chronic obstructive pulmonary disease treated with longacting bronchodilators: two randomised clinical trials. Lancet 374 (2009), 695–703.
    • (2009) Lancet , vol.374 , pp. 695-703
    • Fabbri, L.M.1    Calverley, P.M.2    Izquierdo-Alonso, J.L.3    Bundschuh, D.S.4    Brose, M.5    Martinez, F.J.6    Rabe, K.F.7
  • 73
    • 84930844766 scopus 로고    scopus 로고
    • Roflumilast combined with adenosine increases mucosal hydration in human airway epithelial cultures after cigarette smoke exposure
    • Tyrrell, J., Qian, X., Freire, J., Tarran, R., Roflumilast combined with adenosine increases mucosal hydration in human airway epithelial cultures after cigarette smoke exposure. Am J Physiol Lung Cell Mol Physiol 308 (2015), L1068–L1077.
    • (2015) Am J Physiol Lung Cell Mol Physiol , vol.308 , pp. L1068-L1077
    • Tyrrell, J.1    Qian, X.2    Freire, J.3    Tarran, R.4


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