Increased apical Na+ permeability in cystic fibrosis is supported by a quantitative model of epithelial ion transport

Journal of Physiology

Volumn 591, Issue 15, 2013, Pages 3681-3692

  O'Donoghue, Donal L ^a   Dua, Vivek ^a   Moss, Guy W J ^a   Vergani, Paola ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

AMILORIDE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPITHELIAL SODIUM CHANNEL; SODIUM ION;

APICAL MEMBRANE; ARTICLE; CELL MEMBRANE POTENTIAL; HUMAN; HUMAN CELL; HYPERPOLARIZATION; LUNG FIBROSIS; MATHEMATICAL MODEL; NOSE MUCOSA; PRIORITY JOURNAL; PROTEIN DEPLETION; QUANTITATIVE ANALYSIS; SODIUM TRANSPORT;

EID: 84881008900     PISSN: 00223751     EISSN: 14697793     Source Type: Journal    
DOI: 10.1113/jphysiol.2013.253955     Document Type: Article

References (37)

1. Benjamin BA & Johnson EA (1997). A quantitative description of the Na-K-2Cl cotransporter and its conformity to experimental data. Am J Physiol Renal Physiol 273, F473-F482.
2. Chen J-H, Stoltz DA, Karp PH, Ernst SE, Pezzulo AA, Moninger TO, Rector M V, Reznikov LR, Launspach JL, Chaloner K, Zabner J & Welsh MJ (2010). Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. Cell 143, 911-923.
3. Coote K, Atherton-Watson HC, Sugar R, Young A, MacKenzie-Beevor A, Gosling M, Bhalay G, Bloomfield G, Dunstan A, Bridges RJ, Sabater JR, Abraham WM, Tully D, Pacoma R, Schumacher A, Harris J & Danahay H (2009). Camostat attenuates airway epithelial sodium channel function in vivo through the inhibition of a channel-activating protease. J Pharmacol Exp Ther 329, 764-774.
4. Cotton CU (2000). Basolateral potassium channels and epithelial ion transport. Am J Respir Cell Mol Biol 23, 270-272.
5. Cuthbert AW (2011). New horizons in the treatment of cystic fibrosis. Br J Pharmacol 163, 173-183.
6. Davies JC, Alton EWFW & Bush A (2007). Cystic fibrosis. BMJ 335, 1255-1259.
7. Dodge JA, Lewis PA, Stanton M & Wilsher J (2007). Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur Respir J 29, 522-526.
8. Donaldson SH & Boucher RC (2007). Sodium channels and cystic fibrosis. Chest 132, 1631-1636.
9. Duszyk M & French AS (1991). An analytical model of ionic movements in airway epithelial cells. J Theor Biol 151, 231-247.
10. Falkenberg CV & Jakobsson E (2010). A biophysical model for integration of electrical, osmotic, and pH regulation in the human bronchial epithelium. Biophys J 98, 1476-1485.
11. Fischer H, Illek B, Finkbeiner WE & Widdicombe JH (2007). Basolateral Cl channels in primary airway epithelial cultures. Am J Physiol Lung Cell Mol Physiol 292, L1432-L1443.
12. Flynn AN, Itani OA, Moninger TO & Welsh MJ (2009). Acute regulation of tight junction ion selectivity in human airway epithelia. Proc Natl Acad Sci U S A 106, 3591-3596.
13. Garcia GJM, Boucher RC & Elston TC (2013). Biophysical model of ion transport across human respiratory epithelia allows quantification of ion permeabilities. Biophys J 104, 716-726.
14. Hartmann T & Verkman AS (1990). Model of ion transport regulation in chloride-secreting airway epithelial cells. Integrated description of electrical, chemical, and fluorescence measurements. Biophys J 58, 391-401.
15. Hille B (2001). Ion Channels of Excitable Membranes, 3rd edn. Sinauer, Sunderland, MA, USA
16. Hofmann T, Stutts MJ, Ziersch A, Rückes C, Weber WM, Knowles MR, Lindemann H & Boucher RC (1998). Effects of topically delivered benzamil and amiloride on nasal potential difference in cystic fibrosis. Am J Respir Crit Care Med 157, 1844-1849.
17. Horisberger J-D (2003). ENaC-CFTR interactions: the role of electrical coupling of ion fluxes explored in an epithelial cell model. Pflugers Arch 445, 522-528.
18. + conductance. Proc Natl Acad Sci U S A 108, 10260-10265.
19. Knowles MR, Gatzy JT & Boucher RC (1983). Relative ion permeability of normal and cystic fibrosis nasal epithelium. J Clin Invest 71, 1410-1417.
20. 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.
21. + absorption analyzed using an electrokinetic model. Invest Ophthalmol Vis Sci 47, 306-316.
22. - secretion in human airway epithelia. Am J Respir Cell Mol Biol 23, 283-289.
23. 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 U S A 91, 5340-5344.
24. Rowe SM, Clancy JP & Wilschanski M (2011). Nasal potential difference measurements to assess CFTR ion channel activity. Methods Mol Biol 741, 69-86.
25. Rowe SM, Miller S & Sorscher EJ (2005). Cystic fibrosis. N Engl J Med 352, 1992-2001.
26. Simmonds NJ, D'Souza L, Roughton M, Alton EWFW, Davies JC & Hodson ME (2011). Cystic fibrosis and survival to 40 years: a study of cystic fibrosis transmembrane conductance regulator function. Eur Respir J 37, 1076-1082.
27. Smith NP & Crampin EJ (2004). Development of models of active ion transport for whole-cell modelling: cardiac sodium-potassium pump as a case study. Prog Biophys Mol Biol 85, 387-405.
28. Sobie EA (2009). Parameter sensitivity analysis in electrophysiological models using multivariable regression. Biophys J 96, 1264-1274.
29. Stutts MJ, Canessa CM, Olsen JC, Hamrick M, Cohn JA, Rossier BC & Boucher RC (1995). CFTR as a cAMP-dependent regulator of sodium channels. Science 269, 847-850.
30. Stutts MJ, Knowles MR, Gatzy JT & Boucher RC (1986). Oxygen consumption and ouabain binding sites in cystic fibrosis nasal epithelium. Pediatr Res 20, 1316-1320.
31. Taylor AL, Goaillard J-M & Marder E (2009). How multiple conductances determine electrophysiological properties in a multicompartment model. J Neurosci 29, 5573-5586.
32. Warren NJ, Tawhai MH & Crampin EJ (2009). A mathematical model of calcium-induced fluid secretion in airway epithelium. J Theor Biol 259, 837-849.
33. Willumsen NJ & Boucher RC (1989). Shunt resistance and ion permeabilities in normal and cystic fibrosis airway epithelia. Am J Physiol Cell Physiol 256, C1054-C1063.
34. Willumsen NJ & Boucher RC (1991a). Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium. Am J Physiol Cell Physiol 261, C332-C341.
35. Willumsen NJ & Boucher RC (1991b). Sodium transport and intracellular sodium activity in cultured human nasal epithelium. Am J Physiol Cell Physiol 261, C319-C331.
36. - pathways in cultured human airway epithelium. Am J Physiol Cell Physiol 256, C1033-C1044.
37. - transport in cultured cystic fibrosis airway epithelium. Am J Physiol Cell Physiol 256, C1045-C1053.