A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease

PLoS ONE

Volumn 7, Issue 6, 2012, Pages

  Sloane, Peter A ^f   Shastry, Suresh ^f   Wilhelm, Andrew ^a   Courville, Clifford ^a   Tang, Li Ping ^f   Backer, Kyle ^f   Levin, Elina ^f   Raju, S Vamsee ^f   Li, Yao ^f   Mazur, Marina ^f   Byan Parker, Suzanne ^d   Grizzle, William ^d   Sorscher, Eric J ^a,c,f   Dransfield, Mark T ^a,e,f   Rowe, Steven M ^a,b,c,e,f  

^a University of Alabama at Birmingham   (United States)

^b University of Alabama at Birmingham   (United States)

^c UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

^d University of Alabama at Birmingham   (United States)

^e UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

^f UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

IVACAFTOR; MESSENGER RNA; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ADULT; AGED; ARTICLE; BRONCHUS MUCOSA; CHLORIDE TRANSPORT; CIGARETTE SMOKING; CONCENTRATION RESPONSE; CONTROLLED STUDY; DRUG EFFICACY; EPITHELIUM CELL; FEMALE; HUMAN; HUMAN CELL; HUMAN TISSUE; ION TRANSPORT; LUNG DISEASE; MALE; PROTEIN EXPRESSION; PROTEIN FUNCTION; PROTEIN TARGETING;

ADULT; AGED; AGED, 80 AND OVER; AMINOPHENOLS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DEMOGRAPHY; EPITHELIAL CELLS; FEMALE; HUMANS; ION TRANSPORT; LUNG DISEASES; MALE; MEMBRANE POTENTIALS; MIDDLE AGED; MUCUS; QUINOLONES; SMOKING;

EID: 84863090252     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0039809     Document Type: Article

References (55)

1. Rowe SM, Miller S, Sorscher EJ, (2005) Cystic fibrosis. N Engl J Med 352: 1992-2001.
2. Amaral MD, Kunzelmann K, (2007) Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis. Trends Pharmacol Sci 28: 334-341.
3. Varga K, Goldstein RF, Jurkuvenaite A, Chen L, Matalon S, et al. (2008) Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones. Biochem J 410: 555-564.
4. Houghton AM, Quintero PA, Perkins DL, Kobayashi DK, Kelley DG, et al. (2006) Elastin fragments drive disease progression in a murine model of emphysema. J Clin Invest 116: 753-759.
5. Kellermayer R, Szigeti R, Keeling KM, Bedekovics T, Bedwell DM, (2006) Aminoglycosides as potential pharmacogenetic agents in the treatment of Hailey-Hailey disease. J Invest Dermatol 126: 229-231.
6. Barnes PJ, (2004) Small airways in COPD. N Engl J Med 350: 2635-2637.
7. Martinez-Garcia MA, Soler-Cataluna JJ, Donat Sanz Y, Catalan Serra P, Agramunt Lerma M, et al. (2011) Factors associated with bronchiectasis in patients with COPD. Chest 140: 1130-1137.
8. Sethi S, Maloney J, Grove L, Wrona C, Berenson CS, (2006) Airway inflammation and bronchial bacterial colonization in chronic obstructive pulmonary disease. Am J Respir Crit Care Med 173: 991-998.
9. Soler N, Ewig S, Torres A, Filella X, Gonzalez J, et al. (1999) Airway inflammation and bronchial microbial patterns in patients with stable chronic obstructive pulmonary disease. Eur Respir J 14: 1015-1022.
10. Hogg JC, Chu F, Utokaparch S, Woods R, Elliott WM, et al. (2004) The nature of small-airway obstruction in chronic obstructive pulmonary disease. N Engl J Med 350: 2645-2653.
11. Vestbo J, (2002) Epidemiological studies in mucus hypersecretion. Novartis Found Symp 248: 3-12; discussion 12-19, 277-282.
12. Hogg JC, Chu FS, Tan WC, Sin DD, Patel SA, et al. (2007) Survival after lung volume reduction in chronic obstructive pulmonary disease: insights from small airway pathology. Am J Respir Crit Care Med 176: 454-459.
13. Cantin AM, Hanrahan JW, Bilodeau G, Ellis L, Dupuis A, et al. (2006) Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers. Am J Respir Crit Care Med 173: 1139-1144.
14. Kreindler JL, Jackson AD, Kemp PA, Bridges RJ, Danahay H, (2005) Inhibition of chloride secretion in human bronchial epithelial cells by cigarette smoke extract. Am J Physiol Lung Cell Mol Physiol 288: L894-902.
15. Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, et al. (2011) A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. The New England Journal of Medicine 365: 1663-1672.
16. Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, et al. (2010) Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 363: 1991-2003.
17. Van Goor F, Hadida S, Grootenhuis PD, Burton B, Cao D, et al. (2009) Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci U S A 106: 18825-18830.
18. Rowe SM, Dransfield MT, (2010) Lower airway potential difference measurements in COPD subjects: Emerging evidence of acquired CFTR dysfunction. Ped Pulmonol pp. 216-217.
19. Rowe SM, Pyle LC, Jurkevante A, Varga K, Collawn J, et al. (2010) DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers. Pulm Pharmacol Ther 23: 268-278.
20. Jurkuvenaite A, Varga K, Nowotarski K, Kirk KL, Sorscher EJ, et al. (2006) Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis. J Biol Chem 281: 3329-3334.
21. Rowe SM, Varga K, Rab A, Bebok Z, Byram K, et al. (2007) Restoration of W1282X CFTR Activity by Enhanced Expression. Am J Respir Cell Mol Biol 37: 347-356.
22. Bebok Z, Collawn JF, Wakefield J, Parker W, Li Y, et al. (2005) Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers. J Physiol 569: 601-615.
23. Cobb BR, Fan L, Kovacs TE, Sorscher EJ, Clancy JP, (2003) Adenosine receptors and phosphodiesterase inhibitors stimulate Cl- secretion in Calu-3 cells. Am J Respir Cell Mol Biol 29: 410-418.
24. Matsui H, Randell SH, Peretti SW, Davis CW, Boucher RC, (1998) Coordinated clearance of periciliary liquid and mucus from airway surfaces. Journal of Clinical Investigation 102: 1125-1131.
25. Azbell C, Zhang S, Skinner D, Fortenberry J, Sorscher EJ, et al. (2010) Hesperidin stimulates cystic fibrosis transmembrane conductance regulator-mediated chloride secretion and ciliary beat frequency in sinonasal epithelium. Otolaryngology-head and neck surgery: official journal of American Academy of Otolaryngology-Head and Neck Surgery 143: 397-404.
26. Rollins BM, Burn M, Coakley RD, Chambers LA, Hirsh AJ, et al. (2008) A2B adenosine receptors regulate the mucus clearance component of the lung's innate defense system. Am J Respir Cell Mol Biol 39: 190-197.
27. Leidy NK, Rennard SI, Schmier J, Jones MK, Goldman M, (2003) The breathlessness, cough, and sputum scale: the development of empirically based guidelines for interpretation. Chest 124: 2182-2191.
28. Solomon GM, Konstan MW, Wilschanski M, Billings J, Sermet-Gaudelus I, et al. (2010) An international randomized multicenter comparison of nasal potential difference techniques. Chest 138: 919-928.
29. van der Toorn M, Rezayat D, Kauffman HF, Bakker SJ, Gans RO, et al. (2009) Lipid-soluble components in cigarette smoke induce mitochondrial production of reactive oxygen species in lung epithelial cells. Am J Physiol Lung Cell Mol Physiol 297: L109-114.
30. Sarir H, Mortaz E, Karimi K, Kraneveld AD, Rahman I, et al. (2009) Cigarette smoke regulates the expression of TLR4 and IL-8 production by human macrophages. J Inflamm (Lond) 6: 12.
31. Mortaz E, Kraneveld AD, Smit JJ, Kool M, Lambrecht BN, et al. (2009) Effect of cigarette smoke extract on dendritic cells and their impact on T-cell proliferation. PLoS One 4: e4946.
32. Kasai A, Hiramatsu N, Hayakawa K, Yao J, Maeda S, et al. (2006) High levels of dioxin-like potential in cigarette smoke evidenced by in vitro and in vivo biosensing. Cancer Res 66: 7143-7150.
33. Savitski AN, Mesaros C, Blair IA, Cohen NA, Kreindler JL, (2009) Secondhand smoke inhibits both Cl- and K+ conductances in normal human bronchial epithelial cells. Respir Res 10: 120.
34. Innes AL, Woodruff PG, Ferrando RE, Donnelly S, Dolganov GM, et al. (2006) Epithelial mucin stores are increased in the large airways of smokers with airflow obstruction. Chest 130: 1102-1108.
35. Rowe SM, Clancy JP, Boyle M, Van Goor F, Ordonez C, et al. (2010) Parallel effects of VX-770 on transepithelial potential difference in vitro and in vivo. Journal of Cystic Fibrosis 9 (Supplement).
36. Welsh MJ, (1983) Cigarette smoke inhibition of ion transport in canine tracheal epithelium. J Clin Invest 71: 1614-1623.
37. Cohn JA, Friedman KJ, Noone PG, Knowles MR, Silverman LM, et al. (1998) Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis. N Engl J Med 339: 653-658.
38. Sharer N, Schwarz M, Malone G, Howarth A, Painter J, et al. (1998) Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N Engl J Med 339: 645-652.
39. Kerem E, Hirawat S, Armoni S, Yaakov Y, Shoseyov D, et al. (2008) Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial. Lancet 372: 719-727.
40. Marchand E, Verellen-Dumoulin C, Mairesse M, Delaunois L, Brancaleone P, et al. (2001) Frequency of cystic fibrosis transmembrane conductance regulator gene mutations and 5T allele in patients with allergic bronchopulmonary aspergillosis. Chest 119: 762-767.
41. Miller PW, Hamosh A, Macek M Jr, Greenberger PA, MacLean J, et al. (1996) Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in allergic bronchopulmonary aspergillosis. American journal of human genetics 59: 45-51.
42. Howard MT, Shirts BH, Petros LM, Flanigan KM, Gesteland RF, et al. (2000) Sequence specificity of aminoglycoside-induced stop condon readthrough: potential implications for treatment of Duchenne muscular dystrophy. Ann Neurol 48: 164-169.
43. Pignatti PF, Bombieri C, Benetazzo M, Casartelli A, Trabetti E, et al. (1996) CFTR gene variant IVS8-5T in disseminated bronchiectasis. Am J Hum Genet 58: 889-892.
44. Girodon E, Cazeneuve C, Lebargy F, Chinet T, Costes B, et al. (1997) CFTR gene mutations in adults with disseminated bronchiectasis. Eur J Hum Genet 5: 149-155.
45. Knowles MR, Durie PR, (2002) What is cystic fibrosis? N Engl J Med 347: 439-442.
46. Wilschanski M, Dupuis A, Ellis L, Jarvi K, Zielenski J, et al. (2006) Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials. Am J Respir Crit Care Med 174: 787-794.
47. Koblizek V, Tomsova M, Cermakova E, Papousek P, Pracharova S, et al. (2011) Impairment of nasal mucociliary clearance in former smokers with stable chronic obstructive pulmonary disease relates to the presence of a chronic bronchitis phenotype. Rhinology 49: 397-406.
48. Rennolds J, Butler S, Maloney K, Boyaka PN, Davis IC, et al. (2010) Cadmium regulates the expression of the CFTR chloride channel in human airway epithelial cells. Toxicological sciences: an official journal of the Society of Toxicology 116: 349-358.
49. Guimbellot JS, Fortenberry JA, Siegal GP, Moore B, Wen H, et al. (2008) Role of oxygen availability in CFTR expression and function. Am J Respir Cell Mol Biol 39: 514-521.
50. Rab A, Bartoszewski R, Jurkuvenaite A, Wakefield J, Collawn JF, et al. (2007) Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression. Am J Physiol Cell Physiol 292: C756-766.
51. Bodas M, Min T, Mazur S, Vij N, (2010) Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema. J Immunol 186: 602-613.
52. Hogg JC, Chu FS, Tan WC, Sin DD, Patel SA, et al. (2007) Survival after lung volume reduction in chronic obstructive pulmonary disease: insights from small airway pathology. American journal of respiratory and critical care medicine 176: 454-459.
53. Zheng JP, Kang J, Huang SG, Chen P, Yao WZ, et al. (2008) Effect of carbocisteine on acute exacerbation of chronic obstructive pulmonary disease (PEACE Study): a randomised placebo-controlled study. Lancet 371: 2013-2018.
54. Decramer M, Rutten-van Molken M, Dekhuijzen PN, Troosters T, van Herwaarden C, et al. (2005) Effects of N-acetylcysteine on outcomes in chronic obstructive pulmonary disease (Bronchitis Randomized on NAC Cost-Utility Study, BRONCUS): a randomised placebo-controlled trial. Lancet 365: 1552-1560.
55. Fahy JV, Dickey BF, (2010) Airway mucus function and dysfunction. N Engl J Med 363: 2233-2247.