Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways

Proceedings of the National Academy of Sciences of the United States of America

Volumn 110, Issue 40, 2013, Pages 15973-15978

  Garland, Alaina L ^a   Walton, William G ^b   Coakley, Raymond D ^a   Tan, Chong D ^a   Gilmore, Rodney C ^a   Hobbs, Carey A ^a   Tripathy, Ashutosh ^a   Clunes, Lucy A ^a   Bencharit, Sompop ^c   Stutts, M Jackson ^a   Betts, Laurie ^b   Redinbo, Matthew R ^a,b   Tarran, Robert ^a  

^a UNIVERSITY OF NORTH CAROLINA SCHOOL OF MEDICINE   (United States)

^b University of North Carolina at Chapel Hill   (United States)

^c UNIVERSITY OF NORTH CAROLINA   (United States)

Author keywords

Bacterial permeability increasing protein; COPD innate defense; Ion channels

Indexed keywords

EPITHELIAL SODIUM CHANNEL;

ADULT; ALKALINIZATION; ARTICLE; CONTROLLED STUDY; CRYSTAL STRUCTURE; CYSTIC FIBROSIS; DEHYDRATION; HUMAN; LUNG DISEASE; PH; PRIORITY JOURNAL; SODIUM ABSORPTION;

BACTERIAL PERMEABILITY-INCREASING PROTEIN; COPD INNATE DEFENSE; ION CHANNELS;

ADULT; ANALYSIS OF VARIANCE; CELLS, CULTURED; CRYSTALLIZATION; CYSTIC FIBROSIS; DEHYDRATION; EPITHELIAL SODIUM CHANNELS; GENE KNOCKDOWN TECHNIQUES; GLYCOPROTEINS; HUMANS; HYDROGEN-ION CONCENTRATION; MODELS, MOLECULAR; MUCUS; NORTH CAROLINA; PHOSPHOPROTEINS; RESPIRATORY MUCOSA;

EID: 84885084821     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1311999110     Document Type: Article

References (36)

1. Eaton DC, Malik B, Bao HF, Yu L, Jain L (2010) Regulation of epithelial sodium channel trafficking by ubiquitination. Proc Am Thorac Soc 7(1):54-64.
2. Rossier BC, Stutts MJ (2009) Activation of the epithelial sodium channel (ENaC) by serine proteases. Annu Rev Physiol 71:361-379.
3. Kleyman TR, Carattino MD, Hughey RP (2009) ENaC at the cutting edge: Regulation of epithelial sodium channels by proteases. J Biol Chem 284(31):20447-20451.
4. 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(5):487-493.
5. Caldwell RA, Boucher RC, Stutts MJ (2004) Serine protease activation of near-silent epithelial Na+ channels. Am J Physiol Cell Physiol 286(1):C190-C194.
6. Rossier BC, Pradervand S, Schild L, Hummler E (2002) Epithelial sodium channel and the control of sodium balance: Interaction between genetic and environmental factors. Annu Rev Physiol 64:877-897.
7. Althaus M (2013) ENaC inhibitors and airway re-hydration in cystic fibrosis: State of the art. Curr Mol Pharmacol 6(1):3-12.
8. Kimura T, et al. (2011) Deletion of the ubiquitin ligase Nedd4L in lung epithelia causes cystic fibrosis-like disease. Proc Natl Acad Sci USA 108(8):3216-3221.
9. 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(11): L1141-L1146.
10. Knowles M, Gatzy J, Boucher R (1981) Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med 305(25):1489-1495.
11. Boucher RC, Stutts MJ, Knowles MR, Cantley L, Gatzy JT (1986) Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. J Clin Invest 78(5):1245-1252.
12. Gentzsch M, et al. (2010) The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na+ channel. J Biol Chem 285(42): 32227-32232.
13. Berdiev BK, Qadri YJ, Benos DJ (2009) Assessment of the CFTR and ENaC association. Mol Biosyst 5(2):123-127.
14. Gaillard EA, et al. (2010) Regulation of the epithelial Na+ channel and airway surface liquid volume by serine proteases. Pflugers Arch 460(1):1-17.
15. Garcia-Caballero A, et al. (2009) SPLUNC1 regulates airway surface liquid volume by protecting ENaC from proteolytic cleavage. Proc Natl Acad Sci USA 106(27): 11412-11417.
16. Bingle CD, Craven CJ (2002) PLUNC: A novel family of candidate host defence proteins expressed in the upper airways and nasopharynx. Hum Mol Genet 11(8):937-943.
17. Bingle L, et al. (2007) Differential epithelial expression of the putative innate immune molecule SPLUNC1 in cystic fibrosis. Respir Res 8:79.
18. Roxo-Rosa M, et al. (2006) Proteomic analysis of nasal cells from cystic fibrosis patients and non-cystic fibrosis control individuals: Search for novel biomarkers of cystic fibrosis lung disease. Proteomics 6(7):2314-2325.
19. Tarran R, Trout L, Donaldson SH, Boucher RC (2006) Soluble mediators, not cilia, determine airway surface liquid volume in normal and cystic fibrosis superficial airway epithelia. J Gen Physiol 127(5):591-604.
20. Poulsen JH, Fischer H, Illek B, Machen TE (1994) Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator. Proc Natl Acad Sci USA 91(12):5340-5344.
21. Coakley RD, et al. (2003) Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium. Proc Natl Acad Sci USA 100(26):16083-16088.
22. Pezzulo AA, et al. (2012) Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature 487(7405):109-113.
23. Jayaraman S, Joo NS, Reitz B, Wine JJ, Verkman AS (2001) Submucosal gland secretions in airways from cystic fibrosis patients have normal [Na(+)] and pH but elevated viscosity. Proc Natl Acad Sci USA 98(14):8119-8123.
24. Quinton PM (2008) Cystic fibrosis: Impaired bicarbonate secretion and mucoviscidosis. Lancet 372(9636):415-417.
25. Chmiel JF, Davis PB (2003) State of the art: Why do the lungs of patients with cystic fibrosis become infected and why can't they clear the infection? Respir Res 4:8.
26. Derichs N, Jin BJ, Song Y, Finkbeiner WE, Verkman AS (2011) Hyperviscous airway periciliary and mucous liquid layers in cystic fibrosis measured by confocal fluorescence photobleaching. FASEB J 25(7):2325-2332.
27. Matsui H, et al. (1998) Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 95(7): 1005-1015.
28. Quinton PM (2010) Role of epithelial HCO3 transport in mucin secretion: Lessons from cystic fibrosis. Am J Physiol Cell Physiol 299(6):C1222-C1233.
29. Knowles MR, Boucher RC (2002) Mucus clearance as a primary innate defense mechanism for mammalian airways. J Clin Invest 109(5):571-577.
30. Boren EJ, Teuber SS, Gershwin ME (2008) A review of non-cystic fibrosis pediatric bronchiectasis. Clin Rev Allergy Immunol 34(2):260-273.
31. Mousa HM, Woodley FW (2012) Gastroesophageal reflux in cystic fibrosis: Current understandings of mechanisms and management. Curr Gastroenterol Rep 14(3): 226-235.
32. Fong P (2012) CFTR-SLC26 transporter interactions in epithelia. Biophys Rev 4(2): 107-116.
33. Stutts MJ, et al. (1995) CFTR as a cAMP-dependent regulator of sodium channels. Science 269(5225):847-850.
34. Bartlett JA, et al. (2011) PLUNC: A multifunctional surfactant of the airways. Biochem Soc Trans 39(4):1012-1016.
35. Liu Y, et al. (2013) SPLUNC1/BPIFA1 contributes to pulmonary host defense against Klebsiella pneumoniae respiratory infection. Am J Pathol 182(5):1519-1531.
36. Coakley RD, et al. (2008) 17beta-Estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia. J Clin Invest 118(12):4025-4035.