Introduction to Section I: Overview of Approaches to Study Cystic Fibrosis Pathophysiology

Methods in Molecular Biology

Volumn 742, Issue , 2011, Pages 3-14

  Clunes, Mark T ^a   Boucher, Richard C ^b  

^a ST GEORGE S UNIVERSITY   (Grenada)

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

Author keywords

Airway surface liquid; defective ion transport; dehydration; mucus layer; respiratory infection; water transport

Indexed keywords

TRANSMEMBRANE CONDUCTANCE REGULATOR; WATER;

AIRWAY REMODELING; ANIMAL; ARTICLE; BACTERIAL INFECTION; BIOFILM; CYSTIC FIBROSIS; DEHYDRATION; GENETICS; HUMAN; INFLAMMATION; LUNG; METABOLISM; METHODOLOGY; MICROBIOLOGY; MOUSE; MUCOCILIARY CLEARANCE; MUCUS; MUTATION; NEUTROPHIL CHEMOTAXIS; OSMOTIC PRESSURE; PATHOPHYSIOLOGY; TRANSPORT AT THE CELLULAR LEVEL;

AIRWAY REMODELING; ANIMALS; BACTERIAL INFECTIONS; BIOFILMS; BIOLOGICAL TRANSPORT; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DEHYDRATION; HUMANS; INFLAMMATION; LUNG; MICE; MUCOCILIARY CLEARANCE; MUCUS; MUTATION; NEUTROPHIL INFILTRATION; OSMOTIC PRESSURE; RESEARCH DESIGN; WATER;

EID: 80052539092     PISSN: 10643745     EISSN: 19406029     Source Type: Book Series    
DOI: 10.1007/978-1-61779-120-8_1     Document Type: Chapter

References (36)

1. Rich, D. P., Anderson, M. P., Gregory, R. J., Cheng, S. H., Paul, S., Jefferson, D. M., McCann, J. D., Klinger, K. W., Smith, A. E., and Welsh, M. J. (1990) Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells. Nature 347, 358–363.
2. Matsui, H., Verghese, M. W., Kesimer, M., Schwab, U. E., Randell, S. H., Sheehan, J. K., Grubb, B. R., and Boucher, R. C. (2005) Reduced 3-dimensional motility in dehydrated airway mucus prevents neutrophil capture and killing bacteria on airway epithelial surfaces. J Immunol 175, 1090– 1099.
3. Bjarnsholt, T., Jensen, P. O., Fiandaca, M. J., Pedersen, J., Hansen, C. R., Andersen, C. B., Pressler, T., Givskov, M., and Hoiby, N. (2009) Pseudomonas aeruginosa biofilms in the respiratory tract of cystic fibrosis patients. Pediatr Pulmonol 44, 547–558.
4. Donaldson, S. H., Bennett, W. D., Zeman, K. L., Knowles, M. R., Tarran, R., and Boucher, R. C. (2006) Mucus clearance and lung function in cystic fibrosis with hypertonic saline. NEnglJMed354, 241–250.
5. Elkins, M. R., Robinson, M., Rose, B. R., Harbour, C., Moriarty, C. P., Marks, G. B., Belousova, E. G., Xuan, W., Bye, P. T. P. and for the National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group (2006) A controlled trial of long-term Inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 354, 229–240.
6. Engelhardt, J. F., Yankaskas, J. R., Ernst, S. A., Yang, Y., Marino, C. R., Boucher, R. C., Cohn, J. A., and Wilson, J. M. (1992) Sub mucosal glands are the predominant site of CFTR expression in human bronchus. Nat Genet 2, 240–247.
7. Kreda, S. M., Mall, M., Mengos, A., Rochelle, L., Yankaskas, J., Riordan, J. R., and Boucher, R. C. (2005) Characterization of wild-type and {Delta}F508 cystic fibrosis transmembrane regulator in human respiratory epithelia. MolBiolCell16, 2154–2167.
8. – channels with altered pore properties. Nature 362, 160–164.
9. Denning, G. M., Anderson, M. P., Amara, J. F., Marshall, J., Smith, A. E., and Welsh, M. J. (1992) Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive. Nature 358, 761–764.
10. Wang, F., Zeltwanger, S., Hu, S., and Hwang, T. C. (2000) Deletion of phenylalanine 508 causes attenuated phosphorylationdependent activation of CFTR chloride channels. J Physiol (Lond) 524 Pt 3, 637–648.
11. Heda, G. D., Tanwani, M., and Marino, C. R. (2001) The Delta F508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells. Am JPhysiol280, C166–C174.
12. Wilson, S. M., Olver, R. E., and Walters, D. V. (2007) Developmental regulation of lumenal lung fluid and electrolyte transport. Respir Physiol Neurobiol 159, 247–255.
13. Kallapur, S. G., and Ikegami, M. (2006) Physiological consequences of intrauterine insults. Paediatr Respir Rev 7, 110–116.
14. Ornoy, A., Arnon, J., Katznelson, D., Granat, M., Caspi, B., and Chemke, J. (1987) Pathological confirmation of cystic fibrosis in the fetus following prenatal diagnosis. Am J Med Genet 28, 935–947.
15. Mason, S. J., Paradiso, A. M., and Boucher, R. C. (1991) Regulation of transepithelial ion transport and intracellular calcium by extracellular adenosine triphosphate in human normal and cystic fibrosis airway epithelium. Br J Pharmacol 103, 1649–1656.
16. Barker, P. M., and Gatzy, J. T. (1998) Effects of adenosine, ATP, and UTP on chloride secretion by epithelia explanted from fetal rat lung. Pediatr Res 43, 652–659.
17. Knowles, M., Gatzy, J., and Boucher, R. (1983) Relative ion permeability of normal and cystic fibrosis nasal epithelium. J Clin Invest 71, 1410–1417.
18. Knowles, M., Gatzy, J., and Boucher, R. (1981) Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. NEnglJMed305, 1489–1495.
19. Wiszniewski, L., Jornot, L., Dudez, T., Pagano, A., Rochat, T., Lacroix, J. S., Suter, S., and Chanson, M. (2006) Long-term cultures of polarized airway epithelial cells from patients with cystic fibrosis. Am J Respir Cell Mol Biol 34, 39–48.
20. Chambers, L. A., Rollins, B. M., and Tarran, R. (2007) Liquid movement across the surface epithelium of large airways. Respir Physiol Neurobiol 159, 256–270.
21. Kerem, E., Bistritzer, T., Hanukoglu, A., Hofmann, T., Zhou, Z., Bennett, W., MacLaughlin, E., Barker, P., Nash, M., Quittell, L., et al. (1999) Pulmonary epithelial sodium channel dysfunction and excess airway liquid in pseudohypoaldosteronism. N Engl J Med 341, 156–162.
22. Johansson, M. E., Thomsson, K. A., and Hansson, G. C. (2009) Proteomic analyses of the two mucus layers of the colon barrier reveal that their main component, the Muc2 mucin, is strongly bound to the Fcgbp protein. JProteomeRes8, 3549–3557.
23. Button, B., and Boucher, R. C. (2008) Role of mechanical stress in regulating airway surface hydration and mucus clearance rates. Respir Physiol Neurobiol 163, 189–201.
24. Zuo, P., Picher, M., Okada, S. F., Lazarowski, E. R., Button, B., Boucher, R. C., and Elston, T. C. (2008) Mathematical model of nucleotide regulation on airway epithelia. Implications for airway homeostasis. JBiol Chem 283, 26805–26819.
25. 2+ in individual small cells using fluorescence Cell Calcium 6, 145–157. microscopy with dual excitation wavelengths.
26. 2+ release and 643–646. influx in polarized epithelia. Nature 377,
27. A., and Boucher, R. C. (2003) The 27. Ribeiro, C. M., Paradiso, A. M., Livraghi, mitochondrial barriers segregate agonistinduced calcium-dependent functions in human airway epithelia. J Gen Physiol 122, 377–387.
28. Stutts, M. J., Canessa, C. M., Olsen, J. C., Hamrick, M., Cohn, J. A., Rossier, B. C., and Boucher, R. C. (1995) CFTR as a cAMPdependent regulator of sodium channels. Science 269, 847–850.
29. Berdiev, B. K., Cormet-Boyaka, E., Tousson, A., Qadri, Y. J., Oosterveld-Hut, C. M., Sorscher, E. J., Lukacs, G. L., et al. H. M., Hong, J. S., Gonzales, P. A., Fuller, (2007) Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer. JBiolChem 282, 36481–36488.
30. Ma, H. P., Saxena, S., and Warnock, D. G. (2002) Anionic phospholipids regulate native and expressed epithelial 7641–7644. sodium channel (ENaC). JBiolChem277,
31. Yamagata, T., Yamagata, Y., Masse, C., Tessier, M. C., Brochiero, E., Dagenais, A., and Berthiaume, Y. (2005) Modulation of expression by protein kinase C in rat alveolar Na+ transport and epithelial sodium channel 977–987. epithelial cells. Can J Physiol Pharmacol 83,
32. Gibson, R. L., Burns, J. L., and Ramsey, B. W. (2003) Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med 168, 918–951.
33. Treis, D., Livraghi, A., Schubert, S., Zhou, 33. Mall, M. A., Harkema, J. R., Trojanek, J. B., Z., Kreda, S. M., Tilley, S. L., Hudson, E. J., chitis and emphysema in beta-epithelial Na+ et al. (2008) Development of chronic bron channel-overexpressing mice. Am J Respir Crit Care Med 177, 730–742.
34. Livraghi, A., Grubb, B. R., Hudson, E. J., Wilkinson, K. J., Sheehan, J. K., Mall, M. A., O’Neal, W. K., Boucher, R. C., and Randell, S. H. (2009) Airway and lung pathology due to mucosal surface dehydration in ß-Epithelial Na+ channeloverexpressing mice: role of TNF-alpha and IL-4R-alpha signaling, influence of neonatal development, and limited efficacy of glucocorticoid treatment. J Immunol 182, 4357–4367.
35. Rogers, C. S., Abraham, W. M., Brogden, K. A., Engelhardt, J. F., Fisher, J. T., McCray, P. B., Jr., McLennan, G., Meyerholz, D. K., Namati, E., Ostedgaard, L. S., et al. (2008) The porcine lung as a potential model for cys tic fibrosis. Am J Physiol Lung Cell Mol Physiol 295, L240–L263.
36. Van Goor, F., Hadida, S., Grootenhuis, P. D., Burton, B., Cao, D., Neuberger, T., Turnbull, A., Singh, A., Joubran, J., Hazlewood, A., 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.