Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs

Journal of Clinical Investigation

Volumn 120, Issue 9, 2010, Pages 3161-3166

  Joo, Nam Soo ^a   Cho, Hyung Ju ^a   Khansaheb, Monal ^a   Wine, Jeffrey J ^a  

^a STANFORD UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CARBACHOL; FORSKOLIN; SUBSTANCE P; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; CYSTIC FIBROSIS; EXPERIMENTAL PIG; MUCUS SECRETION; NEWBORN; NONHUMAN; PRIORITY JOURNAL; PROTEIN DEFICIENCY; STOMACH SECRETION; TRACHEA MUCOSA;

ANIMALS; ANIMALS, NEWBORN; BODY FLUIDS; CARBACHOL; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPITHELIAL CELLS; EXOCRINE GLANDS; FORSKOLIN; RESPIRATORY SYSTEM; SUBSTANCE P; SUS SCROFA; TRACHEA;

EID: 77956354814     PISSN: 00219738     EISSN: 15588238     Source Type: Journal    
DOI: 10.1172/JCI43466     Document Type: Article

References (30)

1. Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med. 2005;352(19):1992-2001.
2. Rogers CS, et al. Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer. J Clin Invest. 2008;118(4):1571-1577.
3. Rogers CS, et al. The porcine lung as a potential model for cystic fibrosis. Am J Physiol Lung Cell Mol Physiol. 2008;295(2):L240-L263.
4. Rogers CS, et al. Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. Science. 2008;321(5897):1837-1841.
5. Stoltz DA, et al. Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. Sci Transl Med. 2010;2(29):29ra31.
6. Joo NS, Irokawa T, Wu JV, Robbins RC, Whyte RI, Wine JJ. Absent secretion to vasoactive intestinal peptide in cystic fibrosis airway glands. J Biol Chem. 2002;277(52):50710-50715.
7. Choi JY, et al. Synergistic airway gland mucus secretion in response to vasoactive intestinal peptide and carbachol is lost in cystic fibrosis. J Clin Invest. 2007;117(10):3118-3127.
8. Choi JY, et al. Substance P stimulates human airway submucosal gland secretion mainly via a CFTR-dependent process. J Clin Invest. 2009;119(5):1189-2000.
9. Jayaraman S, Joo NS, Reitz B, Wine JJ, Verkman AS. Submucosal gland secretions in airways from cystic fibrosis patients have normal [Na+] and pH but elevated viscosity. Proc Natl Acad Sci U S A. 2001;98(14):8119-8123.
10. Wine JJ, Joo NS. Submucosal glands and airway defense. Proceedings of the American Thoracic Society. 2004;1(1):47-53.
11. Salinas D, et al. Submucosal gland dysfunction as a primary defect in cystic fibrosis. FASEB J. 2005;19(3):431-433.
12. Joo NS, Irokawa T, Robbins RC, Wine JJ. Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands. J Biol Chem. 2006;281(11):7392-7398.
13. Meyerholz DK, et al. Loss of CFTR function produces abnormalities in tracheal development in neonatal pigs and young children [published online ahead of print July 9, 2010]. Am J Respir Crit Care Med. doi:10.1164/rccm.201004- 0643OC.
14. Joo NS, Wu JV, Krouse ME, Saenz Y, Wine JJ. Optical method for quantifying rates of mucus secretion from single submucosal glands. Am J Physiol Lung Cell Mol Physiol. 2001;281(2):L458-L468.
15. Joo NS, Saenz Y, Krouse ME, Wine JJ. Mucus secretion from single submucosal glands of pig. Stimulation by carbachol and vasoactive intestinal peptide. J Biol Chem. 2002;277(31):28167-28175.
16. Trout L, Corboz MR, Ballard ST. Mechanism of substance P-induced liquid secretion across bronchial epithelium. Am J Physiol Lung Cell Mol Physiol. 2001;281(3):L639-L645.
17. Lamb JP, Sparrow MP. Three-dimensional mapping of sensory innervation with substance p in porcine bronchial mucosa: comparison with human airways. Am J Respir Crit Care Med. 2002;166(9):1269-1281.
18. Berschneider HM, et al. Altered intestinal chloride transport in cystic fibrosis. FASEB J. 1988;2(10):2625-2629.
19. Taylor CJ, Baxter PS, Hardcastle J, Hardcastle PT. Failure to induce secretion in jejunal biopsies from children with cystic fibrosis. Gut. 1988;29(7):957-962.
20. Bijman J, et al. Chloride transport in the cystic fibrosis enterocyte. Adv Exp Med Biol. 1991;290:287-294.
21. Ousingsawat J, Martins JR, Schreiber R, Rock JR, Harfe BD, Kunzelmann K. Loss of TMEM16A causes a defect in epithelial Ca2+ dependent chloride transport. J Biol Chem. 2009;284(42):28698-28703.
22. Lee RJ, Foskett JK. Mechanisms of Ca2+-stimulated fluid secretion by porcine bronchial submucosal gland serous acinar cells. Am J Physiol Lung Cell Mol Physiol. 2009;298(2):L210-L231.
23. Boucher RC. Airway surface dehydration in cystic fibrosis: pathogenesis and therapy. Annu Rev Med. 2007;58:157-170.
24. Ianowski JP, Choi JY, Wine JJ, Hanrahan JW. Mucus secretion by single tracheal submucosal glands from normal and cystic fibrosis transmembrane conductance regulator CFTR knock-out mice. J Physiol. 2007;580(pt 1):301-314.
25. Wine JJ. Parasympathetic control of airway submucosal glands: Central reflexes and the airway intrinsic nervous system. Auton Neurosci. 2007;133(1):35-54.
26. Ballard ST, Spadafora D. Fluid secretion by submucosal glands of the tracheobronchial airways. Respir Physiol Neurobiol. 2007;159(3):271-277.
27. Joo NS, Lee DJ, Winges KM, Rustagi A, Wine JJ. Regulation of antiprotease and antimicrobial protein secretion by airway submucosal gland serous cells. J Biol Chem. 2004;279(37):38854-38860.
28. Clarke LL, et al. Abnormal Paneth cell granule dissolution and compromised resistance to bacterial colonization in the intestine of CF mice. Am J Physiol Gastrointest Liver Physiol. 2004;286(6):G1050-G1058.
29. Conner GE, Wijkstrom-Frei C, Randell SH, Fernandez VE, Salathe M. The lactoperoxidase system links anion transport to host defense in cystic fibrosis. FEBS Lett. 2007;581(2):271-278.
30. Moskwa P, et al. A novel host defense system of airways is defective in cystic fibrosis. Am J Respir Crit Care Med. 2006;175(2):174-183.