Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease

Cell

Volumn 95, Issue 7, 1998, Pages 1005-1015

  Matsui, Hirotoshi ^a   Grubb, Barbara R ^a   Tarran, Robert ^a   Randell, Scott H ^a   Gatzy, John T ^a   Davis, C William ^a   Boucher, Richard C ^a  

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

Author keywords

[No Author keywords available]

Indexed keywords

TRANSMEMBRANE CONDUCTANCE REGULATOR;

ANIMAL CELL; ARTICLE; CELL COMPARTMENTALIZATION; CYSTIC FIBROSIS; EPITHELIUM CELL; ION TRANSPORT; LUNG DISEASE; MUCOCILIARY TRANSPORT; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN DEPLETION; PROTEIN EXPRESSION; RESPIRATORY EPITHELIUM; RESPIRATORY TRACT INFECTION;

EID: 0032433707     PISSN: 00928674     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0092-8674(00)81724-9     Document Type: Article

References (48)

1. Baker, E., Jeunemaitre, X., Portal, A.J., Grimbert, P., Markandu, N., Persu, A., Corvol, P., and MacGregor, G. (1998). Abnormalities of nasal potential difference measurement in Liddle's syndrome. J. Clin. Invest. 102, 10-14.
2. Ballard, S.T., and Gatzy, J.T. (1991). Volume flow across the alveolar epithelium of adult rat lung. J. Appl. Physiol. 70, 1665-1676.
3. Blake, J. (1975). On the movement of mucus in the lung. J. Biomech. 8, 179-190.
4. Boucher, R.C. (1994a). Human airway ion transport. Part 1. Am. J. Respir. Grit. Care Med. 150, 271-281.
5. Boucher, R.C. (1994b). Human airway ion transport. Part 2. Am. J. Respir. Crit. Care Med. 150, 581-593.
6. Davis, P.B., Drumm, M., and Konstan, M.W. (1996). Cystic fibrosis. Am. J. Respir. Crit. Care Med. 154, 1229-1256.
7. Davis, C.W., Matsui, H., Tarran, R., and Boucher, R.C. (1998). Osmotic water permeability of well-differentiated normal and CF airway epithelial cell cultures: cellular and paracellular paths as measured by confocal microscopy. Pediatr. Pulmonol. Suppl. 17, 247-248 (Abstract).
8. Farinas, J., Kneen, M., Moore, M., and Verkman, A.S. (1997). Plasma membrane water permeability of cultured cells and epithelia measured by light microscopy with spatial filtering. J. Gen. Physiol. 110, 283-296.
9. Gilljam, H., Ellin, A., and Strandvik, B. (1989). Increased bronchial chloride concentration in cystic fibrosis. Scand. J. Clin. Lab. Invest. 49, 121-124.
10. Goldman, M.J., Anderson, G.M., Stolzenberg, E.D., Kari, U.P., Zasloff, M., and Wilson, J.M. (1997). Human beta-defensin-1 is a salt-sensitive antibiotic that is inactivated in cystic fibrosis. Cell 88, 553-560.
11. Grubb, B.R., Schiretz, F.R., and Boucher, R.C. (1997). Volume transport across tracheal and bronchial airway epithelia in a tubular culture system. Am. J. Physiol. 273, C21-C29.
12. Hull, J., Skinner, W., Robertson, C., and Phelan, P. (1998). Elemental content of airway surface liquid from infants with cystic fibrosis. Am. J. Respir. Crit. Care Med. 157, 10-14.
13. Imundo, L., Barasch, J., Prince, A., and Al-Awqati, Q. (1995). Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. Proc. Natl. Acad. Sci. USA 92, 3019-3023.
14. Jiang, C., Finkbeiner, W.E., Widdicombe, J.H., McCray, P.B., Jr., and Miller, S.S. (1993). Altered fluid transport across airway epithelium in cystic fibrosis. Science 262, 424-427.
15. Johnson, J.G., Foy, C., Robinson, J., Boucher, R.C., and Knowles, M.R. (1998). Bronchial airway surface liquid (ASL) ion composition in normal subjects [Abstract]. Ped. Pulmonol. Suppl. 17, 241.
16. Joris, L, Dab, I., and Quinton, P.M. (1993). Elemental composition of human airway surface liquid in healthy and diseased airways. Am. Rev. Respir. Dis. 148, 1633-1637.
17. Kerem, E., Bistrizer, T., Hanukoglu, A., MacLaughlin, E., Boucher, R., and Knowles, M. (1997). Respiratory disease in patients with the systemic form of pseudohypoaldosteronism type I. Pediatr. Pulmonol. Suppl. 14, 78-79 (Abstract).
18. Knowles, M.R., Robinson, J.M., Wood, R.E., Pue, C.A., Mentz, W.M., Wager, G.C., Gatzy, J.T., and Boucher, R.C. (1997). Ion composition of airway surface liquid of patients with cystic fibrosis as compared to normal and disease-control subjects. J. Clin. Invest. 100, 2588-2595.
19. Kohler, D., App, E., Schmitz-Schumann, M., Wuertemberger, G., and Matthys, H. (1986). Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibroses. Eur. J. Respir. Dis. Suppl. 146, 319-326.
20. Konstan, M.W., and Berger, M. (1997). Current understanding of the inflammatory process in cystic fibrosis: onset and etiology. Pediatr. Pulmonol. 24, 137-142.
21. Lam, J., Chan, R., Lam, K., and Costerton, J.W. (1980). Production of mucoid microcolonies by Pseudomonas aeruginosa within infected lungs in cystic fibrosis. Infect. Immun. 28, 546-556.
22. Levison, H., Mindorff, C.M., Chao, J., Turner, J.A.P., Sturgess, J.M., and Stringer, D. A. (1983). Pathophysiology of the ciliary motility syndromes. Eur. J. Respir. Dis. Suppl. Suppl. 127, 102-116.
23. Matsui, H., Randell, S.H., Peretti, S.W., Davis, C.W., and Boucher, R.C. (1998). Coordinated clearance of periciliary liquid and mucus from airway surfaces. J. Clin. Invest. 102, 1125-1131.
24. Nelson, J.W., Tredgett, M.W., Sheehan, J.K., Thornton, D.J., Notman, D., and Govan, J.R. (1990). Mucinophilic and chemotactic properties of Pseudomonas aeruginosa in relation to pulmonary colonization in cystic fibrosis. Infect. Immun. 58, 1489-1495.
25. Noone, P.G., Bennett, W.D., Zeman, K.L., Regnis, J.A., Carson, J.L., Boucher, R.C., and Knowles, M.R. (1996). Effects on cough clearance of aerosolized uridine-5′-triphosphate +/-amiloride in patients with primary ciliary dyskinesia. Am. J. Respir. Crit. Care Med. 153, A530 (Abstract).
26. Novotny, J.A., and Jakobsson, E. (1996). Computational studies of ion-water flux coupling in the airway epithelium. II. Role of specific transport mechanisms. Am. J. Physiol. 270, C1764-C1772.
27. Pier, G.B., Grout, M., Zaidi, T.S., Olsen, J.C., Johnson, L.G., Yankaskas, J.R., and Goldberg, J.B. (1996). Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections. Science 271, 64-67.
28. Plotkowski, M.C., Zahm, J.M.,Tournier, J.M., and Puchelle, E. (1992). Pseudomonas aeruginosa adhesion to normal and injured respiratory mucosa. Mem. Inst. Oswaldo Cruz 87, Suppl. 5, 61-68.
29. Puchelle, E., de Bentzmann, S., and Zahm, J.M. (1995). Physical and functional properties of airway secretions in cystic fibrosis-therapeutic approaches. Respiration 62, Suppl. 1, 2-12.
30. Quinton, P.M. (1990). Cystic fibrosis: a disease in electrolyte transport. FASEB J. 4, 2709-2717.
31. Quinton, P.M. (1994). Viscosity versus composition in airway pathology [editorial]. Am. J. Respir. Crit. Care Med. 149, 6-7.
32. Regnis, J.A., Robinson, M., Bailey, D.L., Cook, P., Hooper, P., Chan, H.K., Gonda, I., Bautovich, G., and Bye, P.T. (1994). Mucociliary clearance in patients with cystic fibrosis and in normal subjects. Am. J. Respir. Crit. Care Med. 150, 66-71.
33. Satir, P. (1990). The physiology of cilia and mucociliary interactions. Annu. Rev. Physiol. 52, 137-155.
34. Scherer, P.W. (1981). Mucus transport by cough. Chest 80 (Suppl.), 830-833.
35. Sims, D.E., and Home, M.M. (1997). Heterogeneity of the composition and thickness of tracheal mucus in rats. Am. J. Physiol. 273, L1036-L1041.
36. Smith, J.J., and Welsh, M.J. (1993). Fluid and electrolyte transport by cultured human airway epithelia. J. Clin. Invest. 91, 1590-1597.
37. Smith, J.J., Karp, P.H., and Welsh, M.J. (1994). Defective fluid transport by cystic fibrosis airway epithelia. J. Clin. Invest. 93, 1307-1311.
38. Smith, J.J., Travis, S.M., Greenberg, E.P., and Welsh, M.J. (1996). Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell 85, 229-236.
39. + absorption and absence of pulmonary disease in the airways of patients with Liddle's syndrome: potential role of CFTR. Pediatr. Pulmonol. Suppl. 17, 217 (Abstract).
40. Tsang, K.W.T., Rutman, A., Tanaka, E., Lund, V., Dewar, A., Cole, P.J., and Wilson, R. (1994). Interaction of Pseudomonas aeruginosa with human respiratory mucosa in vitro. Eur. Respir. J. 7, 1746-1753.
41. Ulrich, M., Herbert, S., Berger, J., Bellon, G., Louis, D., Muenker, G., and Doering, G. (1998). Localization of Staphylococcus aureus in infected airways of patients with cystic fibrosis and in a cell culture model of S. aureus adherence. Am. J. Respir. Cell Mol. Biol. 19, 83-91.
42. Vishwanath, S., and Ramphal, R. (1984). Adherence of Pseudomonas aeruginosa to human tracheobronchial mucin. Infect. Immun. 45, 197-202.
43. Wanner, A., Salathe, M., and O'Riordan, T.G. (1996). Mucociliary clearance in the airways [State of the Art]. Am. J. Respir. Crit. Care Med. 154, 1868-1902.
44. Widdicombe, J.G. (1997). Airway surface liquid: concepts and measurements. In Airway Mucus: Basic Mechanisms and Clinical Perspectives, D.F. Rogers and M.I. Lethem, eds. (Basel: Birkhauser), pp. 1-17.
45. Willumsen, N.J., Davis, C.W., and Boucher, R.C. (1989). Intracellular Cl activity and cellular Cl pathways in cultured human airway epithelium. Am. J. Physiol. 256, C1033-C1044.
46. Zabner, J., Smith, J.J., Karp, P.M., Widdicombe, J.H., and Welsh, M.J. (1998). Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro. Mol. Cell 2, 397-403.
47. Zahm, J.M., Pierrot, D., Vaquez-Girod, S., Duvivier, C., King, M., and Puchelle, E. (1989). The role of mucus sol phase in clearance by simulated cough. Biorheology 26, 747-752.
48. Zuelzer, W.W., and Newton, W.A., Jr. (1949). The pathogenesis of fibrocystic disease of the pancreas. A study of 36 cases with special reference to the pulmonary lesions. Pediatrics 4, 53-69.