The yin and yang of cystic fibrosis transmembrane conductance regulator function: Implications for chronic lung disease

American Journal of Respiratory and Critical Care Medicine

Volumn 187, Issue 2, 2013, Pages 120-122

  Vij, Neeraj ^a   Downey, Gregory P ^b,c,d  

^a JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b DEPARTMENT OF MEDICINE   (United States)

^c UNIVERSITY OF COLORADO SCHOOL OF MEDICINE   (United States)

^d UNIVERSITY OF COLORADO   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CD2 ANTIGEN; CD4 ANTIGEN; CD8 ANTIGEN; CIGARETTE SMOKE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; LEUKOCYTE ELASTASE; PROTEINASE; PROTEINASE ACTIVATED RECEPTOR 1;

ADAPTIVE IMMUNITY; CHRONIC LUNG DISEASE; CHRONIC OBSTRUCTIVE LUNG DISEASE; CYSTIC FIBROSIS; EDITORIAL; HUMAN; INNATE IMMUNITY; PRIORITY JOURNAL; PROTEIN EXPRESSION; PSEUDOMONAS AERUGINOSA;

ANIMALS; CALPAIN; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; HUMANS; LEUKOCYTE ELASTASE; MALE;

EID: 84872563157     PISSN: 1073449X     EISSN: 15354970     Source Type: Journal    
DOI: 10.1164/rccm.201211-2011ED     Document Type: Editorial

References (33)

1. Mueller C, Braag SA, Keeler A, Hodges C, Drumm M, Flotte TR. Lack of cystic fibrosis transmembrane conductance regulator in CD31 lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses. Am J Respir Cell Mol Biol 2011;44:922-929.
2. Bonfield TL, Hodges CA, Cotton CU, Drumm ML. Absence of the cystic fibrosis transmembrane regulator (Cftr) from myeloid-derived cells slows resolution of inflammation and infection. J Leukoc Biol 2012;92: 1111-1122.
3. Bodas M, Min T, Mazur S, Vij N. Critical modifier role of membranecystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema. J Immunol 2011; 186:602-613.
4. Bajmoczi M, Gadjeva M, Alper SL, Pier GB, Golan DE. Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa. Am J Physiol Cell Physiol 2009; 297:C263-C277.
5. Di A, Brown ME, Deriy LV, Li C, Szeto FL, Chen Y, Huang P, Tong J, Naren AP, Bindokas V, et al. CFTR regulates phagosome acidification in macrophages and alters bactericidal activity.Nat Cell Biol 2006;8:933-944.
6. Bomberger JM, Ye S, Maceachran DP, Koeppen K, Barnaby RL, O'Toole GA, Stanton BA. A Pseudomonas aeruginosa toxin that hijacks the host ubiquitin proteolytic system. PLoS Pathog 2011;7:e1001325.
7. Le Gars M, Descamps D, Roussel D, Saussereau E, Guillot L, Ruffin M, Tabary O, Hong S-S, Boulanger P, Paulis M, et al. Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo. Am J Respir Crit Care Med 2013;187:170-179.
8. Bodas M, Min T, Vij N. Critical role of CFTR-dependent lipid rafts in cigarette smoke-induced lung epithelial injury. Am J Physiol Lung Cell Mol Physiol 2011;300:L811-L820.
9. Cantin AM, Hanrahan JW, Bilodeau G, Ellis L, Dupuis A, Liao J, Zielenski J, Durie P. Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers. Am J Respir Crit Care Med 2006;173:1139-1144.
10. Clunes LA, Davies CM, Coakley RD, Aleksandrov AA, Henderson AG, Zeman KL, Worthington EN, Gentzsch M, Kreda SM, Cholon D, et al. Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration. FASEB J 2012;26:533-545.
11. Sloane PA, Shastry S, Wilhelm A, Courville C, Tang LP, Backer K, Levin E, Raju SV, Li Y, Mazur M, et al. A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease. PLoS ONE 2012;7:e39809.
12. Teichgraber V, Ulrich M, Endlich N, Riethmuller J, Wilker B, De Oliveira-Munding CC, van Heeckeren AM, Barr ML, von Kurthy G, Schmid KW, et al. Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis. Nat Med 2008;14: 382-391.
13. Bartoszewski R, Rab A, Twitty G, Stevenson L, Fortenberry J, Piotrowski A, Dumanski JP, Bebok Z. The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response. J Biol Chem 2008;283:12154-12165.
14. Rab A, Bartoszewski R, Jurkuvenaite A, Wakefield J, Collawn JF, Bebok Z. Endoplasmic reticulum stress and the unfolded protein response regulate genomic cystic fibrosis transmembrane conductance regulator expression. Am J Physiol Cell Physiol 2007;292:C756-C766.
15. Harrington MA, Kopito RR. Cysteine residues in the nucleotide binding domains regulate the conductance state of CFTR channels. Biophys J 2002;82:1278-1292.
16. Nathan C. Neutrophils and immunity: challenges and opportunities. Nat Rev Immunol 2006;6:173-182.
17. Flannagan RS, Jaumouille V, Grinstein S. The cell biology of phagocytosis. Annu Rev Pathol 2012;7:61-98.
18. Hokari R, Miura S. Neutrophil elastase in colitis: more than a marker of disease activity? J Gastroenterol 2006;41:395-396.
19. Abraham E. Neutrophils and acute lung injury. Crit Care Med 2003;31: S195-S199.
20. Zemans RL, Colgan SP, Downey GP. Transepithelial migration of neutrophils: mechanisms and implications for acute lung injury. Am J Respir Cell Mol Biol 2009;40:519-535.
21. Mykles DL. Proteinase families and their inhibitors. Methods Cell Biol 2001;66:247-287.
22. Turk B, Turk du SA, Turk V. Protease signalling: the cutting edge. EMBO J 2012;31:1630-1643.
23. Page-McCaw A, Ewald AJ, Werb Z. Matrix metalloproteinases and the regulation of tissue remodelling. Nat Rev Mol Cell Biol 2007;8: 221-233.
24. Sternlicht MD, Werb Z. How matrix metalloproteinases regulate cell behavior. Annu Rev Cell Dev Biol 2001;17:463-516.
25. Suzuki T, Yamashita C, Zemans RL, Briones N, Van Linden A, Downey GP. Leukocyte elastase induces lung epithelial apoptosis via a PAR-1-, NF-kappaB-, and p53-dependent pathway. Am J Respir Cell Mol Biol 2009;41:742-755.
26. Albertine KH, Soulier MF, Wang Z, Ishizaka A, Hashimoto S, Zimmerman GA, Matthay MA, Ware LB. Fas and fas ligand are upregulated in pulmonary edema fluid and lung tissue of patients with acute lung injury and the acute respiratory distress syndrome. Am J Pathol 2002;161:1783-1796.
27. Matute-Bello G, Liles WC, Steinberg KP, Kiener PA, Mongovin S, Chi EY, Jonas M, Martin TR. Soluble Fas ligand induces epithelial cell apoptosis in humans with acute lung injury (ARDS). J Immunol 1999; 163:2217-2225.
28. Shapiro SD, Goldstein NM, Houghton AM, Kobayashi DK, Kelley D, Belaaouaj A. Neutrophil elastase contributes to cigarette smokeinduced emphysema in mice. Am J Pathol 2003;163:2329-2335.
29. Doring G, Frank F, Boudier C, Herbert S, Fleischer B, Bellon G. Cleavage of lymphocyte surface antigens CD2, CD4, and CD8 by polymorphonuclear leukocyte elastase and cathepsin G in patients with cystic fibrosis. J Immunol 1995;154:4842-4850.
30. Tosi MF, Zakem H. Surface expression of Fc gamma receptor III (CD16) on chemoattractant-stimulated neutrophils is determined by both surface shedding and translocation from intracellular storage compartments. J Clin Invest 1992;90:462-470.
31. Vandivier RW, Fadok VA, Hoffmann PR, Bratton DL, Penvari C, Brown KK, Brain JD, Accurso FJ, Henson PM. Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasis. J Clin Invest 2002;109: 661-670.
32. Caldwell RA, Boucher RC, Stutts MJ. Neutrophil elastase activates near-silent epithelial Na1 channels and increases airway epithelial Na1 transport. Am J Physiol Lung Cell Mol Physiol 2005;288:L813- L819.
33. Adebamiro A, Cheng Y, Rao US, Danahay H, Bridges RJ. A segment of gamma ENaC mediates elastase activation of Na1 transport. J Gen Physiol 2007;130:611-629.