Characterization of Nasal Potential Difference in cftr Knockout and F508del-CFTR Mice

PLoS ONE

Volumn 8, Issue 3, 2013, Pages

  Saussereau, Emilie Lyne ^a,b,c   Roussel, Delphine ^b   Diallo, Siradiou ^b   Debarbieux, Laurent ^a   Edelman, Aleksander ^b   Sermet Gaudelus, Isabelle ^b  

^a INSTITUT PASTEUR   (France)

^b INSERM   (France)

^c SORBONNE UNIVERSITÉ   (France)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; SODIUM ION;

ARTICLE; CONTROLLED STUDY; CYSTIC FIBROSIS; DEPOLARIZATION; ELECTROPHYSIOLOGY; FEMALE; HYPERPOLARIZATION; ION TRANSPORT; KNOCKOUT MOUSE; MALE; MEASUREMENT REPEATABILITY; MOUSE; NASAL POTENTIAL DIFFERENCE; NONHUMAN; PERFUSION; PROTEIN FUNCTION; RESPIRATORY TRACT PARAMETERS; SODIUM TRANSPORT; WILD TYPE;

ANIMALS; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DISEASE MODELS, ANIMAL; FEMALE; HUMANS; MALE; MEMBRANE POTENTIALS; MICE; MICE, KNOCKOUT; NASAL MUCOSA; REPRODUCIBILITY OF RESULTS;

EID: 84874687785     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0057317     Document Type: Article

References (31)

1. Ratjen F, Döring G, (2003) Cystic fibrosis. Lancet 361: 681-689 doi:10.1016/S0140-6736(03)12567-6.
2. Stutts MJ, Canessa CM, Olsen JC, Hamrick M, Cohn JA, et al. (1995) CFTR as a cAMP-dependent regulator of sodium channels. Science 269: 847-850.
3. Amaral M, (2004) CFTR and chaperones. Journal of Molecular Neuroscience 23: 41-48 doi:10.1385/JMN:23:1-2:041.
4. Robert R, Carlile GW, Liao J, Balghi H, Lesimple P, et al. (2010) Correction of the Delta phe508 cystic fibrosis transmembrane conductance regulator trafficking defect by the bioavailable compound glafenine. Mol Pharmacol 77: 922-930 doi:10.1124/mol.109.062679.
5. Van Goor F, Straley KS, Cao D, González J, Hadida S, et al. (2006) Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am J Physiol Lung Cell Mol Physiol 290: L1117-1130 doi:10.1152/ajplung.00169.2005.
6. Colas J, Faure G, Saussereau E, Trudel S, Rabeh WM, et al. (2011) Disruption of cytokeratin-8 interaction with F508del-CFTR corrects its functional defect. Human Molecular Genetics. Available: http://www.ncbi.nlm.nih.gov/pubmed/22038833. Accessed 2011 Dec 5.
7. Tamanini A, Borgatti M, Finotti A, Piccagli L, Bezzerri V, et al. (2011) Trimethylangelicin reduces IL-8 transcription and potentiates CFTR function. Am J Physiol Lung Cell Mol Physiol 300: L380-390 doi:10.1152/ajplung.00129.2010.
8. Grubb BR, Boucher RC, (1999) Pathophysiology of gene-targeted mouse models for cystic fibrosis. Physiological Reviews 79: 193.
9. Goubau C, Wilschanski M, Skalická V, Lebecque P, Southern KW, et al. (2009) Phenotypic characterisation of patients with intermediate sweat chloride values: towards validation of the European diagnostic algorithm for cystic fibrosis. Thorax64: 683-691 doi:10.1136/thx.2008.104752.
10. Sermet-Gaudelus I, Boeck KD, Casimir GJ, Vermeulen F, Leal T, et al. (2010) Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis. Am J Respir Crit Care Med 182: 1262-1272 doi:10.1164/rccm.201001-0137OC.
11. Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, et al. (2010) Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. New England Journal of Medicine 363: 1991-2003.
12. Cartiera MS, Ferreira EC, Caputo C, Egan ME, Caplan MJ, et al. (2010) Partial correction of cystic fibrosis defects with PLGA nanoparticles encapsulating curcumin. Mol Pharm 7: 86-93 doi:10.1021/mp900138a.
13. Lubamba B, Lebacq J, Lebecque P, Vanbever R, Leonard A, et al. (2009) Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice. Am J Respir Crit Care Med 179: 1022-1028 doi:10.1164/rccm.200901-0049OC.
14. Barker PM, (2005) Effect of macrolides on in vivo ion transport across cystic fibrosis nasal epithelium. American Journal of Respiratory and Critical Care Medicine 171: 868-871 doi:10.1164/rccm.200311-1508OC.
15. Brady KG, Kelley TJ, Drumm ML, (2001) Examining basal chloride transport using the nasal potential difference response in a murine model. Am J Physiol Lung Cell Mol Physiol 281: L1173-1179.
16. Grubb BR, Rogers TD, Boucher RC, Ostrowski LE, (2009) Ion transport across CF and normal murine olfactory and ciliated epithelium. American Journal of Physiology- Cell Physiology 296: C1301.
17. Lubamba B, Lecourt H, Lebacq J, Lebecque P, De Jonge H, et al. (2008) Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis. Am J Respir Crit Care Med 177: 506-515 doi:10.1164/rccm.200703-344OC.
18. Griesenbach U, Smith SN, Farley R, Singh C, Alton EWFW, (2008) Validation of Nasal Potential Difference Measurements in Gut-Corrected CF Knockout Mice. American Journal of Respiratory Cell and Molecular Biology 39: 490-496 doi:10.1165/rcmb.2007-0385OC.
19. Salinas DB, Pedemonte N, Muanprasat C, Finkbeiner WF, Nielson DW, et al. (2004) CFTR involvement in nasal potential differences in mice and pigs studied using a thiazolidinone CFTR inhibitor. American Journal of Physiology- Lung Cellular and Molecular Physiology 287: L936.
20. Parsons DW, Hopkins PJ, Bourne AJ, Boucher RC, Martin AJ, (2000) Airway gene transfer in mouse nasal-airways: importance of identification of epithelial type for assessment of gene transfer. Gene Ther 7: 1810-1815 doi:10.1038/sj.gt.3301317.
21. Sermet-Gaudelus I, Girodon E, Roussel D, Deneuville E, Bui S, et al. (2010) Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis. Thorax65: 539-544 doi:10.1136/thx.2009.123422.
22. Bland JM, Altman DG, (1986) Statistical methods for assessing agreement between two methods of clinical measurement. Lancet 1: 307-310.
23. Zhang Z-R, Zeltwanger S, McCarty NA, (2000) Direct Comparison of NPPB and DPC as Probes of CFTR Expressed in Xenopus Oocytes. Journal of Membrane Biology 175: 35-52 doi:10.1007/s002320001053.
24. Schwiebert EM, Morales MM, Devidas S, Egan ME, Guggino WB, (1998) Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator. Proceedings of the National Academy of Sciences95: 2674-2679.
25. Schwiebert EM, Flotte T, Cutting GR, Guggino WB (1994) Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents. American Journal of Physiology- Cell Physiology 266: C1464 -C1477.
26. Bruscia EM, Price JE, Cheng E-C, Weiner S, Caputo C, et al. (2006) Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation. Proc Natl Acad Sci USA 103: 2965-2970 doi:10.1073/pnas.0510758103.
27. Egli M, Duplain H, Lepori M, Cook S, Nicod P, et al. (2004) Defective respiratory amiloride-sensitive sodium transport predisposes to pulmonary oedema and delays its resolution in mice. J Physiol (Lond) 560: 857-865doi:10.1113/jphysiol.2004.066704.
28. Grubb BR, Gabriel SE, Mengos A, Gentzsch M, Randell SH, et al. (2006) SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis. Am J Respir Cell Mol Biol 34: 355-363 doi:10.1165/rcmb.2005-0286OC.
29. Middleton PG, House HH, (2010) Measurement of airway ion transport assists the diagnosis of cystic fibrosis. Pediatr Pulmonol 45: 789-795 doi:10.1002/ppul.21253.
30. Sorensen JB, (2002) The use and misuse of the coefficient of variation in organizational demography research. Sociological Methods & Research 30: 475-491 doi:10.1177/0049124102030004001.
31. Taylor SL, Payton ME, Raun WR, (1999) Relationship between mean yield, coefficient of variation, mean square error, and plot size in wheat field experiments. Communications in Soil Science and Plant Analysis 30: 1439-1447.