The role of CFTR in transepithelial liquid transport in pig alveolar epithelia

American Journal of Physiology - Lung Cellular and Molecular Physiology

Volumn 303, Issue 6, 2012, Pages

  Collawn, James F ^a   Matalon, Sadis ^b  

^a UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

^b UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AMILORIDE; CHLORIDE CHANNEL; CYCLIC AMP; EPITHELIAL SODIUM CHANNEL; TRANSMEMBRANE CONDUCTANCE REGULATOR;

EDITORIAL; HOMEOSTASIS; HUMAN; ION TRANSPORT; LUNG ALVEOLUS CELL TYPE 2; LUNG ALVEOLUS EPITHELIUM; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; RESPIRATORY FAILURE; SWINE; TRANSPORT KINETICS; WATER TRANSPORT;

ANIMALS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EXTRAVASCULAR LUNG WATER; FEMALE; MALE; PNEUMOCYTES;

EID: 84866344890     PISSN: 10400605     EISSN: 15221504     Source Type: Journal    
DOI: 10.1152/ajplung.00216.2012     Document Type: Editorial

References (38)

1. Abu-El-Haija M, Sinkora M, Meyerholz DK, Welsh MJ, McCray PB, Jr, Butler J, and Uc A. An activated immune and inflammatory response targets the pancreas of newborn pigs with cystic fibrosis. Pancreatology 11: 506-515, 2011.
2. Borok Z, Liebler JM, Lubman RL, Foster MJ, Zhou B, Li X, Zabski SM, Kim KJ, Crandall ED. Na transport proteins are expressed by rat alveolar epithelial type I cells. Am J Physiol Lung Cell Mol Physiol 282: L599-L608, 2002.
3. + permeability in cystic fibrosis human primary cell cultures. J Physiol 405: 77-103, 1988.
4. Chen JH, Stoltz DA, Karp PH, Ernst SE, Pezzulo AA, Moninger TO, Rector MV, Reznikov LR, Launspach JL, Chaloner K, Zabner J, Welsh MJ. Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. Cell 143: 911-923, 2010.
5. Collawn JF, Lazrak A, Bebok Z, Matalon S. The CFTR and ENaC debate: how important is ENaC in CF lung disease? Am J Physiol Lung Cell Mol Physiol 302: L1141-L1146, 2012.
6. Davis IC, Xu A, Gao Z, Hickman-Davis JM, Factor P, Sullender WM, Matalon S. Respiratory syncytial virus induces insensitivity to β-adrenergic agonists in mouse lung epithelium in vivo. Am J Physiol Lung Cell Mol Physiol 293: L281-L289, 2007.
7. Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med 173: 475-482, 2006.
8. Engelhardt JF, Zepeda M, Cohn JA, Yankaskas JR, Wilson JM. Expression of the cystic fibrosis gene in adult human lung. J Clin Invest 93: 737-749, 1994.
9. Fang X, Fukuda N, Barbry P, Sartori C, Verkman AS, Matthay MA. Novel role for CFTR in fluid absorption from the distal airspaces of the lung. J Gen Physiol 119: 199-207, 2002.
10. Fang X, Song Y, Hirsch J, Galietta LJ, Pedemonte N, Zemans RL, Dolganov G, Verkman AS, Matthay MA. Contribution of CFTR to apical-basolateral fluid transport in cultured human alveolar epithelial type II cells. Am J Physiol Lung Cell Mol Physiol 290: L242-L249, 2006.
11. Grubb BR, Boucher RC. Pathophysiology of gene-targeted mouse models for cystic fibrosis. Physiol Rev 79: S193-S214, 1999.
12. Hummler E, Barker P, Gatzy J, Beermann F, Verdumo C, Schmidt A, Boucher R, Rossier BC. Early death due to defective neonatal lung liquid clearance in alpha-ENaC-deficient mice. Nat Genet 12: 325-328, 1996.
13. Ismailov II, Awayda MS, Jovov B, Berdiev BK, Fuller CM, Dedman JR, Kaetzel M, Benos DJ. Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator. J Biol Chem 271: 4725-4732, 1996.
14. + conductance. Proc Natl Acad Sci USA 108: 10260-10265, 2011.
15. + channels. Am J Physiol Cell Physiol 275: C1610-C1620, 1998.
16. Johnson MD, Widdicombe JH, Allen L, Barbry P, Dobbs LG. Alveolar epithelial type I cells contain transport proteins and transport sodium, supporting an active role for type I cells in regulation of lung liquid homeostasis. Proc Natl Acad Sci USA 99: 1966-1971, 2002.
17. Kooijman EE, Kuzenko SR, Gong D, Best MD, Folkesson HG. Phosphatidylinositol 4,5-bisphosphate stimulates alveolar epithelial fluid clearance in male and female adult rats. Am J Physiol Lung Cell Mol Physiol 301: L804-L811, 2011.
18. + conductance in cystic fibrosis. Pflügers Arch 431: 1-9, 1995.
19. + conductance by the cystic fibrosis transmembrane conductance regulator. Pflügers Arch 440: 193-201, 2000.
20. Lazrak A, Jurkuvenaite A, Chen L, Keeling KM, Collawn JF, Bedwell DM, Matalon S. Enhancement of alveolar epithelial sodium channel activity with decreased cystic fibrosis transmembrane conductance regulator expression in mouse lung. Am J Physiol Lung Cell Mol Physiol 301: L557-L567, 2011.
21. + transport in ATII cells by cAMP: we agree to disagree and do more experiments. Am J Physiol Lung Cell Mol Physiol 278: L233-L238, 2000.
22. Li X, Comellas AP, Karp PH, Ernst SE, Moninger TO, Gansemer ND, Taft PJ, Pezzulo AA, Rector MV, Rossen N, Stoltz DA, McCray PB Jr, Welsh MJ, Zabner J. CFTR is required for maximal transepithelial liquid transport in pig alveolar epithelia. Am J Physiol Lung Cell Mol Physiol 303: (2) L152-L160, 2012.
23. + absorption produces cystic fibrosis-like lung disease in mice. Nat Med 10: 487-493, 2004.
24. + channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease. Exp Physiol 94: 171-174, 2009.
25. + conductive pathways in alveolar type II pneumocytes. Am J Physiol Lung Cell Mol Physiol 260: L90-L96, 1991.
26. Matthay MA, Folkesson HG, Verkman AS. Salt and water transport across alveolar and distal airway epithelia in the adult lung. Am J Physiol Lung Cell Mol Physiol 270: L487-L503, 1996.
27. Meyerholz DK, Stoltz DA, Namati E, Ramachandran S, Pezzulo AA, Smith AR, Rector MV, Suter MJ, Kao S, McLennan G, Tearney GJ, Zabner J, McCray PB Jr, Welsh MJ. Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children. Am J Respir Crit Care Med 182: 1251-1261, 2010.
28. Meyerholz DK, Stoltz DA, Pezzulo AA, Welsh MJ. Pathology of gastrointestinal organs in a porcine model of cystic fibrosis. Am J Pathol 176: 1377-1389, 2010.
29. Nielsen VG, Duvall MD, Baird MS, Matalon S. cAMP activation of chloride and fluid secretion across the rabbit alveolar epithelium. Am J Physiol Lung Cell Mol Physiol 275: L1127-L1133, 1998.
30. Ostedgaard LS, Meyerholz DK, Chen JH, Pezzulo AA, Karp PH, Rokhlina T, Ernst SE, Hanfland RA, Reznikov LR, Ludwig PS, Rogan MP, Davis GJ, Dohrn CL, Wohlford-Lenane C, Taft PJ, Rector MV, Hornick E, Nassar BS, Samuel M, Zhang Y, Richter SS, Uc A, Shilyansky J, Prather RS, McCray PB Jr, Zabner J, Welsh MJ, Stoltz DA. The DeltaF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs. Sci Transl Med 3: 74ra24, 2011.
31. Rochelle LG, Li DC, Ye H, Lee E, Talbot CR, Boucher RC. Distribution of ion transport mRNAs throughout murine nose and lung. Am J Physiol Lung Cell Mol Physiol 279: L14-L24, 2000.
32. Rogers CS, Abraham WM, Brogden KA, Engelhardt JF, Fisher JT, McCray PB Jr, McLennan G, Meyerholz DK, Namati E, Ostedgaard LS, Prather RS, Sabater JR, Stoltz DA, Zabner J, Welsh MJ. The porcine lung as a potential model for cystic fibrosis. Am J Physiol Lung Cell Mol Physiol 295: L240-L263, 2008.
33. Rubenstein RC, Lockwood SR, Lide E, Bauer R, Suaud L, Grumbach Y. 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, 2011.
34. Sakuma T, Okaniwa G, Nakada T, Nishimura T, Fujimura S, Matthay MA. Alveolar fluid clearance in the resected human lung. Am J Respir Crit Care Med 150: 305-310, 1994.
35. Soukup B, Benjamin A, Orogo-Wenn M, Walters D. Physiological effect of protein kinase C on ENaC-mediated lung liquid regulation in the adult rat lung. Am J Physiol Lung Cell Mol Physiol 302: L133-L139, 2012.
36. Stoltz DA, Meyerholz DK, Pezzulo AA, Ramachandran S, Rogan MP, Davis GJ, Hanfland RA, Wohlford-Lenane C, Dohrn CL, Bartlett JA, Nelson GA, Chang EH, Taft PJ, Ludwig PS, Estin M, Hornick EE, Launspach JL, Samuel M, Rokhlina T, Karp PH, Ostedgaard LS, Uc A, Starner TD, Horswill AR, Brogden KA, Prather RS, Richter SS, Shilyansky J, McCray PB Jr, Zabner J, Welsh MJ. Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. Sci Transl Med 2: 29ra31, 2010.
37. Stutts MJ, Canessa CM, Olsen JC, Hamrick M, Cohn JA, Rossier BC, Boucher RC. CFTR as a cAMP-dependent regulator of sodium channels. Science 269: 847-850, 1995.
38. Yan W, Samaha FF, Ramkumar M, Kleyman TR, Rubenstein RC. Cystic fibrosis transmembrane conductance regulator differentially regulates human and mouse epithelial sodium channels in Xenopus oocytes. J Biol Chem 279: 23183-23192, 2004.