CFTR regulates early pathogenesis of chronic obstructive lung disease in βenac-overexpressing mice

PLoS ONE

Volumn 7, Issue 8, 2012, Pages

  Johannesson, Bjarki ^a,b   Hirtz, Stephanie ^a   Schatterny, Jolanthe ^a   Schultz, Carsten ^a,b   Mall, Marcus A ^a,b  

^a UNIVERSITY OF HEIDELBERG   (Germany)

^b EUROPEAN MOLECULAR BIOLOGY LABORATORY   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE; CYTOKINE; PROTEIN KC; SODIUM; TRANSMEMBRANE CONDUCTANCE REGULATOR; TUMOR NECROSIS FACTOR ALPHA; UNCLASSIFIED DRUG;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL COUNT; CHLORIDE TRANSPORT; CHRONIC OBSTRUCTIVE LUNG DISEASE; CONTROLLED STUDY; CYTOKINE PRODUCTION; DISEASE SEVERITY; ENVIRONMENTAL FACTOR; GENE DELETION; HEREDITY; MOLECULAR PATHOLOGY; MOUSE; NEWBORN; NEWBORN MORTALITY; NONHUMAN; PROTEIN EXPRESSION; PROTEIN FUNCTION; SODIUM ABSORPTION; SODIUM TRANSPORT; SURVIVAL RATE;

AIRWAY OBSTRUCTION; ANIMALS; ANIMALS, NEWBORN; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DISEASE PROGRESSION; EPITHELIAL SODIUM CHANNELS; EPITHELIUM; LUNG; MICE; MICE, INBRED BALB C; MICE, INBRED C57BL; MICE, INBRED CFTR; MICE, TRANSGENIC; PULMONARY DISEASE, CHRONIC OBSTRUCTIVE; RESPIRATORY SYSTEM;

MUS;

EID: 84866627429     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0044059     Document Type: Article

References (50)

1. Rabe KF, Hurd S, Anzueto A, Barnes PJ, Buist SA, et al. (2007) Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease: GOLD executive summary. Am J Respir Crit Care Med 176: 532-555.
2. Silverman EK, (2006) Progress in chronic obstructive pulmonary disease genetics. Proc Am Thorac Soc 3: 405-408.
3. Cookson WO, Moffatt MF, (2011) Genetics of complex airway disease. Proc Am Thorac Soc 8: 149-153.
4. Mall MA, Harkema JR, Trojanek JB, Treis D, Livraghi A, et al. (2008) Development of chronic bronchitis and emphysema in β-epithelial Na+ channel-overexpressing mice. Am J Respir Crit Care Med 177: 730-742.
5. Knowles MR, Boucher RC, (2002) Mucus clearance as a primary innate defense mechanism for mammalian airways. J Clin Invest 109: 571-577.
6. Mall MA, (2008) Role of cilia, mucus, and airway surface liquid in mucociliary dysfunction: lessons from mouse models. J Aerosol Med Pulm Drug Deliv 21: 13-24.
7. Goodman RM, Yergin BM, Landa JF, Golivanux MH, Sackner MA, (1978) Relationship of smoking history and pulmonary function tests to tracheal mucous velocity in nonsmokers, young smokers, ex-smokers, and patients with chronic bronchitis. Am Rev Respir Dis 117: 205-214.
8. Hogg JC, Timens W, (2009) The pathology of chronic obstructive pulmonary disease. Annu Rev Pathol 4: 435-459.
9. Welsh MJ, (1983) Cigarette smoke inhibition of ion transport in canine tracheal epithelium. J Clin Invest 71: 1614-1623.
10. 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.
11. Clunes LA, Davies CM, Coakley RD, Aleksandrov AA, Henderson AG, et al. (2012) Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration. FASEB J 26: 533-545.
12. Sloane PA, Shastry S, Wilhelm A, Courville C, Tang LP, et al. (2012) A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease. PLoS ONE 7: e39809.
13. Wielputz MO, Eichinger M, Zhou Z, Leotta K, Hirtz S, et al. (2011) In vivo monitoring of cystic fibrosis-like lung disease in mice by volumetric computed tomography. Eur Respir J 38: 1060-1070.
14. Zhou L, Dey CR, Wert SE, DuVall MD, Frizzell RA, et al. (1994) Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR. Science 266: 1705-1708.
15. Mall M, Grubb BR, Harkema JR, O'Neal WK, Boucher RC, (2004) Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice. Nat Med 10: 487-493.
16. Zhou Z, Duerr J, Johannesson B, Schubert SC, Treis D, et al. (2011) The ENaC-overexpressing mouse as a model of cystic fibrosis lung disease. J Cyst Fibros 10Suppl 2: S172-S182.
17. Anagnostopoulou P, Dai L, Schatterny J, Hirtz S, Duerr J, et al. (2010) Allergic airway inflammation induces a pro-secretory epithelial ion transport phenotype in mice. Eur Respir J 36: 1436-1447.
18. Mall M, Bleich M, Greger R, Schreiber R, Kunzelmann K, (1998) The amiloride inhibitable Na+ conductance is reduced by CFTR in normal but not in cystic fibrosis airways. J Clin Invest 102: 15-21.
19. Roth EK, Hirtz S, Duerr J, Wenning D, Eichler I, et al. (2011) The K+ channel opener 1-EBIO potentiates residual function of mutant CFTR in rectal biopsies from cystic fibrosis patients. PLoS ONE 6: e24445.
20. Harkema JR, Plopper CG, Hyde DM, St George JA, (1987) Regional differences in quantities of histochemically detectable mucosubstances in nasal, paranasal, and nasopharyngeal epithelium of the bonnet monkey. J Histochem Cytochem 35: 279-286.
21. Zhou Z, Treis D, Schubert SC, Harm M, Schatterny J, et al. (2008) Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in βENaC-overexpressing mice. Am J Respir Crit Care Med 178: 1245-1256.
22. Scherle W, (1970) A simple method for volumetry of organs in quantitative stereology. Mikroskopie 26: 57-60.
23. Dunnill MS, (1962) Quantitative methods in the study of pulmonary pathology. Thorax 17: 320-328.
24. Pfaffl MW, (2001) A new mathematical model for relative quantification in real-time RT-PCR. Nucleic Acids Res 29: E45.
25. McNicholas CM, Canessa CM, (1997) Diversity of channels generated by different combinations of epithelial sodium channel subunits. J Gen Physiol 109: 681-692.
26. Mall MA, Button B, Johannesson B, Zhou Z, Livraghi A, et al. (2010) Airway surface liquid volume regulation determines different airway phenotypes in Liddle compared with βENaC-overexpressing mice. J Biol Chem 285: 26945-26955.
27. Rock JR, O'Neal WK, Gabriel SE, Randell SH, Harfe BD, et al. (2009) Transmembrane protein 16A (TMEM16A) is a Ca2+-regulated Cl- secretory channel in mouse airways. J Biol Chem 284: 14875-14880.
28. Stutts MJ, Canessa CM, Olsen JC, Hamrick M, Cohn JA, et al. (1995) CFTR as a cAMP-dependent regulator of sodium channels. Science 269: 847-850.
29. Hopf A, Schreiber R, Mall M, Greger R, Kunzelmann K, (1999) Cystic fibrosis transmembrane conductance regulator inhibits epithelial Na+ channels carrying Liddle's syndrome mutations. J Biol Chem 274: 13894-13899.
30. Livraghi-Butrico A, Grubb BR, Kelly EJ, Wilkinson KJ, Yang H, et al. (2012) Genetically determined heterogeneity of lung disease in a mouse model of airway mucus obstruction. Physiol Genomics 44: 470-484.
31. Mall M, Hipper A, Greger R, Kunzelmann K, (1996) Wild type but not ΔF508 CFTR inhibits Na+ conductance when coexpressed in Xenopus oocytes. FEBS Lett 381: 47-52.
32. Rubenstein RC, Lockwood SR, Lide E, Bauer R, Suaud L, et al. (2011) Regulation of endogenous ENaC functional expression by CFTR and DeltaF508-CFTR in airway epithelial cells. Am J Physiol Lung Cell Mol Physiol 300: L88-L101.
33. Mall MA, (2009) Role of the amiloride-sensitive epithelial Na+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease. Exp Physiol 94: 171-174.
34. Grubb BR, O'Neal WK, Ostrowski LE, Kreda SM, Button B, et al. (2012) Transgenic hCFTR expression fails to correct β-ENaC mouse lung disease. Am J Physiol Lung Cell Mol Physiol 302: L238-L247.
35. Duerr J, Gruner M, Schubert SC, Haberkorn U, Bujard H, et al. (2011) Use of a new-generation reverse tetracycline transactivator system for quantitative control of conditional gene expression in the murine lung. Am J Respir Cell Mol Biol 44: 244-254.
36. Collawn JF, Lazrak A, Bebok Z, Matalon S, (2012) The CFTR and ENaC Debate - How Important is ENaC in CF Lung Disease? Am J Physiol Lung Cell Mol Physiol 302: L1141-6.
37. Welsh MJ, Ramsey BW, Accurso F, Cutting GR (2001) Cystic fibrosis. In: Scriver CR, Beaudet AL, Sly WS, and Valle D, editors. The Metabolic & Molecular Bases of Inherited Disease. New York: McGraw-Hill. 5121-5188.
38. Kunzelmann K, Mall M, (2001) Pharmacotherapy of the ion transport defect in cystic fibrosis. Clin Exp Pharmacol Physiol 28: 857-867.
39. Sly PD, Brennan S, Gangell C, de Klerk N, Murray C, et al. (2009) Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening. Am J Respir Crit Care Med 180: 146-152.
40. Knowles MR, Paradiso AM, Boucher RC, (1995) In vivo nasal potential difference: techniques and protocols for assessing efficacy of gene transfer in cystic fibrosis. Hum Gene Ther 6: 445-455.
41. Boucher RC, (2004) Relationship of airway epithelial ion transport to chronic bronchitis. Proc Am Thorac Soc 1: 66-70.
42. Bodas M, Min T, Mazur S, Vij N, (2011) Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema. J Immunol 186: 602-613.
43. Bodas M, Min T, Vij N, (2011) Critical role of CFTR-dependent lipid rafts in cigarette smoke-induced lung epithelial injury. Am J Physiol Lung Cell Mol Physiol 300: L811-L820.
44. Teichgraber V, Ulrich M, Endlich N, Riethmuller J, Wilker B, et al. (2008) Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis. Nat Med 14: 382-391.
45. Petrache I, Natarajan V, Zhen L, Medler TR, Richter AT, et al. (2005) Ceramide upregulation causes pulmonary cell apoptosis and emphysema-like disease in mice. Nat Med 11: 491-498.
46. 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 USA 106: 18825-18830.
47. 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.
48. 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. N Engl J Med 365: 1663-1672.
49. Becq F, Mall MA, Sheppard DN, Conese M, Zegarra-Moran O, (2011) Pharmacological therapy for cystic fibrosis: from bench to bedside. J Cyst Fibros 10Suppl 2: S129-S145.
50. Peters LL, Robledo RF, Bult CJ, Churchill GA, Paigen BJ, et al. (2007) The mouse as a model for human biology: a resource guide for complex trait analysis. Nat Rev Genet 8: 58-69.