Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis

Science

Volumn 345, Issue 6198, 2014, Pages 818-822

  Hoegger, Mark J ^a   Fischer, Anthony J ^a   McMenimen, James D ^a   Ostedgaard, Lynda S ^a   Tucker, Alex J ^a   Awadalla, Maged A ^a   Moninger, Thomas O ^a   Michalski, Andrew S ^a   Hoffman, Eric A ^a,b   Zabner, Joseph ^a   Stoltz, David A ^a,b   Welsh, Michael J ^a  

^a UNIVERSITY OF IOWA   (United States)

^b IA United States   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BACTERIUM; MUCUS; NUMERICAL MODEL; PIG; SECRETION;

AIRWAY REMODELING; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ANION TRANSPORT; ARTICLE; CHOLINERGIC STIMULATION; CONTROLLED STUDY; CYSTIC FIBROSIS; GENOTYPE; LUNG FIBROSIS; MUCOCILIARY TRANSPORT; MUCUS SECRETION; NEWBORN; NONHUMAN; PARTICLE SIZE; PHENOTYPE; PRIORITY JOURNAL; ANIMAL; DISEASE MODEL; EUKARYOTIC FLAGELLUM; EXOCRINE GLAND; LUNG; METABOLISM; MUCOCILIARY CLEARANCE; MUCUS; PATHOPHYSIOLOGY; PHYSIOLOGY; RESPIRATORY SYSTEM; RESPIRATORY TRACT MUCOSA; SECRETION (PROCESS); SWINE; TRACHEA;

ANION; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; METHACHOLINE CHLORIDE;

ANIMALS; ANIMALS, NEWBORN; ANIONS; CILIA; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DISEASE MODELS, ANIMAL; EXOCRINE GLANDS; LUNG; METHACHOLINE CHLORIDE; MUCOCILIARY CLEARANCE; MUCUS; RESPIRATORY MUCOSA; RESPIRATORY SYSTEM; SWINE; TRACHEA;

EID: 84906089250     PISSN: 00368075     EISSN: 10959203     Source Type: Journal    
DOI: 10.1126/science.1255825     Document Type: Article

References (30)

1. M. J. Welsh, B. W. Ramsey, F. Accurso, G. R. Cutting, in The Metabolic and Molecular Basis of Inherited Disease, C. R. Scriver et al., Eds. (McGraw-Hill, New York, 2001), pp. 5121-5189.
2. A. Wanner, M. Salathe, T. G. O'Riordan, Am. J. Respir. Crit. Care Med. 154, 1868-1902 (1996).
3. M. Robinson, P. T. Bye, Pediatr. Pulmonol. 33, 293-306 (2002).
4. J. J. Wine, N. S. Joo, Proc. Am. Thorac. Soc. 1, 47-53 (2004).
5. R. C. Boucher, J. Intern. Med. 261, 5-16 (2007).
6. D. McShane et al., Eur. Respir. J. 24, 95-100 (2004).
7. J. A. Regnis et al., Am. J. Respir. Crit. Care Med. 150, 66-71 (1994).
8. J. V. Fahy, B. F. Dickey, N. Engl. J. Med. 363, 2233-2247 (2010).
9. D. A. Stoltz et al., Sci. Transl. Med. 2, 29ra31 (2010).
10. M. J. Hoegger et al., Proc. Natl. Acad. Sci. U.S.A. 111, 2355-2360 (2014).
11. S. T. Ballard, D. Spadafora, Respir. Physiol. Neurobiol. 159, 271-277 (2007).
12. J. J. Wine, Auton. Neurosci. 133, 35-54 (2007).
13. J.-H. Chen et al., Cell 143, 911-923 (2010).
14. U. Griesenbach et al., Am. J. Respir. Cell Mol. Biol. 44, 309-315 (2011).
15. N. S. Joo, H. J. Cho, M. Khansaheb, J. J. Wine, J. Clin. Invest. 120, 3161-3166 (2010).
16. D. X. Y. Wu et al., Am. J. Physiol. 274, L388-L395 (1998).
17. A. Billet, J. W. Hanrahan, J. Physiol. 591, 5273-5278 (2013).
18. R. J. Lee, J. K. Foskett, J. Biol. Chem. 287, 38316-38326 (2012).
19. J. F. Engelhardt et al., Nat. Genet. 2, 240-248 (1992).
20. R. J. Lee, J. M. Harlow, M. P. Limberis, J. M. Wilson, J. K. Foskett, J. Gen. Physiol. 132, 161-183 (2008).
21. J. H. Poulsen, H. Fischer, B. Illek, T. E. Machen, Proc. Natl. Acad. Sci. U.S.A. 91, 5340-5344 (1994).
22. S. Moon, M. Singh, M. E. Krouse, J. J. Wine, Am. J. Physiol. 273, L1208-L1219 (1997).
23. L. Trout, J. T. Gatzy, S. T. Ballard, Am. J. Physiol. 275, L1095-L1099 (1998).
24. S. T. Ballard, S. K. Inglis, J. Physiol. 556, 1-10 (2004).
25. P. M. Quinton, Lancet 372, 415-417 (2008).
26. P. M. Quinton, Am. J. Physiol. 299, C1222-C1233 (2010).
27. J. K. Gustafsson et al., J. Exp. Med. 209, 1263-1272 (2012).
28. A. A. Pezzulo et al., Nature 487, 109-113 (2012).
29. M. Cohen-Cymberknoh et al., Chest 145, 738-744 (2014).
30. B. J. Staudinger et al., Am. J. Respir. Crit. Care Med. 189, 812-824 (2014).