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Journal of the Royal Society of Medicine logoLink to Journal of the Royal Society of Medicine
. 1999;92(Suppl 37):23–30. doi: 10.1177/014107689909237s05

The protease-antiprotease battle in the cystic fibrosis lung.

I M Balfour-Lynn 1
PMCID: PMC1297020  PMID: 10472249

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Advances in airway disease research. A workshop on asthma and bronchial inflammation. Monterey, California, May 19-22, 1993. Proceedings. Am J Respir Crit Care Med. 1994 Nov;150(5 Pt 2):S1–105. doi: 10.1164/ajrccm/150.5_Pt_2.S1. [DOI] [PubMed] [Google Scholar]
  2. Armstrong D. S., Grimwood K., Carzino R., Carlin J. B., Olinsky A., Phelan P. D. Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis. BMJ. 1995 Jun 17;310(6994):1571–1572. doi: 10.1136/bmj.310.6994.1571. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Balough K., McCubbin M., Weinberger M., Smits W., Ahrens R., Fick R. The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis. Pediatr Pulmonol. 1995 Aug;20(2):63–70. doi: 10.1002/ppul.1950200203. [DOI] [PubMed] [Google Scholar]
  4. Baumstark J. S., Lee C. T., Luby R. J. Rapid inactivation of alpha1-protease inhibitor (alpha1-antitrypsin) by elastase. Biochim Biophys Acta. 1977 Jun 10;482(2):400–411. doi: 10.1016/0005-2744(77)90254-6. [DOI] [PubMed] [Google Scholar]
  5. Bingle L., Tetley T. D. Secretory leukoprotease inhibitor: partnering alpha 1-proteinase inhibitor to combat pulmonary inflammation. Thorax. 1996 Dec;51(12):1273–1274. doi: 10.1136/thx.51.12.1273. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Birrer P. Consequences of unbalanced protease in the lung: protease involvement in destruction and local defense mechanisms of the lung. Agents Actions Suppl. 1993;40:3–12. doi: 10.1007/978-3-0348-7385-7_1. [DOI] [PubMed] [Google Scholar]
  7. Birrer P., McElvaney N. G., Rüdeberg A., Sommer C. W., Liechti-Gallati S., Kraemer R., Hubbard R., Crystal R. G. Protease-antiprotease imbalance in the lungs of children with cystic fibrosis. Am J Respir Crit Care Med. 1994 Jul;150(1):207–213. doi: 10.1164/ajrccm.150.1.7912987. [DOI] [PubMed] [Google Scholar]
  8. Bonfield T. L., Konstan M. W., Burfeind P., Panuska J. R., Hilliard J. B., Berger M. Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis. Am J Respir Cell Mol Biol. 1995 Sep;13(3):257–261. doi: 10.1165/ajrcmb.13.3.7544594. [DOI] [PubMed] [Google Scholar]
  9. Bonfield T. L., Panuska J. R., Konstan M. W., Hilliard K. A., Hilliard J. B., Ghnaim H., Berger M. Inflammatory cytokines in cystic fibrosis lungs. Am J Respir Crit Care Med. 1995 Dec;152(6 Pt 1):2111–2118. doi: 10.1164/ajrccm.152.6.8520783. [DOI] [PubMed] [Google Scholar]
  10. Canonico A. E., Brigham K. L., Carmichael L. C., Plitman J. D., King G. A., Blackwell T. R., Christman J. W. Plasmid-liposome transfer of the alpha 1 antitrypsin gene to cystic fibrosis bronchial epithelial cells prevents elastase-induced cell detachment and cytokine release. Am J Respir Cell Mol Biol. 1996 Apr;14(4):348–355. doi: 10.1165/ajrcmb.14.4.8600939. [DOI] [PubMed] [Google Scholar]
  11. Cantin A., Bilodeau G., Bégin R. Granulocyte elastase-mediated proteolysis of alpha 1-antitrypsin in cystic fibrosis bronchopulmonary secretions. Pediatr Pulmonol. 1989;7(1):12–17. doi: 10.1002/ppul.1950070105. [DOI] [PubMed] [Google Scholar]
  12. Carver A. S., Dalrymple M. A., Wright G., Cottom D. S., Reeves D. B., Gibson Y. H., Keenan J. L., Barrass J. D., Scott A. R., Colman A. Transgenic livestock as bioreactors: stable expression of human alpha-1-antitrypsin by a flock of sheep. Biotechnology (N Y) 1993 Nov;11(11):1263–1270. doi: 10.1038/nbt1193-1263. [DOI] [PubMed] [Google Scholar]
  13. Costello C. M., O'Connor C. M., Finlay G. A., Shiels P., FitzGerald M. X., Hayes J. P. Effect of nebulised recombinant DNase on neutrophil elastase load in cystic fibrosis. Thorax. 1996 Jun;51(6):619–623. doi: 10.1136/thx.51.6.619. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. De Water R., Willems L. N., Van Muijen G. N., Franken C., Fransen J. A., Dijkman J. H., Kramps J. A. Ultrastructural localization of bronchial antileukoprotease in central and peripheral human airways by a gold-labeling technique using monoclonal antibodies. Am Rev Respir Dis. 1986 May;133(5):882–890. [PubMed] [Google Scholar]
  15. Dean T. P., Dai Y., Shute J. K., Church M. K., Warner J. O. Interleukin-8 concentrations are elevated in bronchoalveolar lavage, sputum, and sera of children with cystic fibrosis. Pediatr Res. 1993 Aug;34(2):159–161. doi: 10.1203/00006450-199308000-00010. [DOI] [PubMed] [Google Scholar]
  16. Delacourt C., Le Bourgeois M., D'Ortho M. P., Doit C., Scheinmann P., Navarro J., Harf A., Hartmann D. J., Lafuma C. Imbalance between 95 kDa type IV collagenase and tissue inhibitor of metalloproteinases in sputum of patients with cystic fibrosis. Am J Respir Crit Care Med. 1995 Aug;152(2):765–774. doi: 10.1164/ajrccm.152.2.7633740. [DOI] [PubMed] [Google Scholar]
  17. Delcroix M., Kurz X., Walckiers D., Demedts M., Naeije R. High incidence of primary pulmonary hypertension associated with appetite suppressants in Belgium. Eur Respir J. 1998 Aug;12(2):271–276. doi: 10.1183/09031936.98.12020271. [DOI] [PubMed] [Google Scholar]
  18. DiMango E., Ratner A. J., Bryan R., Tabibi S., Prince A. Activation of NF-kappaB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells. J Clin Invest. 1998 Jun 1;101(11):2598–2605. doi: 10.1172/JCI2865. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Döring G., Høiby N. Longitudinal study of immune response to Pseudomonas aeruginosa antigens in cystic fibrosis. Infect Immun. 1983 Oct;42(1):197–201. doi: 10.1128/iai.42.1.197-201.1983. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Fick R. B., Jr, Robbins R. A., Squier S. U., Schoderbek W. E., Russ W. D. Complement activation in cystic fibrosis respiratory fluids: in vivo and in vitro generation of C5a and chemotactic activity. Pediatr Res. 1986 Dec;20(12):1258–1268. doi: 10.1203/00006450-198612000-00014. [DOI] [PubMed] [Google Scholar]
  21. Gillissen A., Birrer P., McElvaney N. G., Buhl R., Vogelmeier C., Hoyt R. F., Jr, Hubbard R. C., Crystal R. G. Recombinant secretory leukoprotease inhibitor augments glutathione levels in lung epithelial lining fluid. J Appl Physiol (1985) 1993 Aug;75(2):825–832. doi: 10.1152/jappl.1993.75.2.825. [DOI] [PubMed] [Google Scholar]
  22. Jagger K. S., Bahner D. R., Warren R. L. Protease phenotypes of Pseudomonas aeruginosa isolated from patients with cystic fibrosis. J Clin Microbiol. 1983 Jan;17(1):55–59. doi: 10.1128/jcm.17.1.55-59.1983. [DOI] [PMC free article] [PubMed] [Google Scholar]
  23. Khan T. Z., Wagener J. S., Bost T., Martinez J., Accurso F. J., Riches D. W. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med. 1995 Apr;151(4):1075–1082. doi: 10.1164/ajrccm/151.4.1075. [DOI] [PubMed] [Google Scholar]
  24. Koller D. Y., Urbanek R., Götz M. Increased degranulation of eosinophil and neutrophil granulocytes in cystic fibrosis. Am J Respir Crit Care Med. 1995 Aug;152(2):629–633. doi: 10.1164/ajrccm.152.2.7633718. [DOI] [PubMed] [Google Scholar]
  25. Konstan M. W., Berger M. Current understanding of the inflammatory process in cystic fibrosis: onset and etiology. Pediatr Pulmonol. 1997 Aug;24(2):137–161. doi: 10.1002/(sici)1099-0496(199708)24:2<137::aid-ppul13>3.0.co;2-3. [DOI] [PubMed] [Google Scholar]
  26. Konstan M. W., Hilliard K. A., Norvell T. M., Berger M. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am J Respir Crit Care Med. 1994 Aug;150(2):448–454. doi: 10.1164/ajrccm.150.2.8049828. [DOI] [PubMed] [Google Scholar]
  27. Konstan M. W., Walenga R. W., Hilliard K. A., Hilliard J. B. Leukotriene B4 markedly elevated in the epithelial lining fluid of patients with cystic fibrosis. Am Rev Respir Dis. 1993 Oct;148(4 Pt 1):896–901. doi: 10.1164/ajrccm/148.4_Pt_1.896. [DOI] [PubMed] [Google Scholar]
  28. Mahadeva R., Westerbeek R. C., Perry D. J., Lovegrove J. U., Whitehouse D. B., Carroll N. R., Ross-Russell R. I., Webb A. K., Bilton D., Lomas D. A. Alpha1-antitrypsin deficiency alleles and the Taq-I G-->A allele in cystic fibrosis lung disease. Eur Respir J. 1998 Apr;11(4):873–879. doi: 10.1183/09031936.98.11040873. [DOI] [PubMed] [Google Scholar]
  29. McElvaney N. G., Doujaiji B., Moan M. J., Burnham M. R., Wu M. C., Crystal R. G. Pharmacokinetics of recombinant secretory leukoprotease inhibitor aerosolized to normals and individuals with cystic fibrosis. Am Rev Respir Dis. 1993 Oct;148(4 Pt 1):1056–1060. doi: 10.1164/ajrccm/148.4_Pt_1.1056. [DOI] [PubMed] [Google Scholar]
  30. McElvaney N. G., Hubbard R. C., Birrer P., Chernick M. S., Caplan D. B., Frank M. M., Crystal R. G. Aerosol alpha 1-antitrypsin treatment for cystic fibrosis. Lancet. 1991 Feb 16;337(8738):392–394. doi: 10.1016/0140-6736(91)91167-s. [DOI] [PubMed] [Google Scholar]
  31. McElvaney N. G., Nakamura H., Birrer P., Hébert C. A., Wong W. L., Alphonso M., Baker J. B., Catalano M. A., Crystal R. G. Modulation of airway inflammation in cystic fibrosis. In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor. J Clin Invest. 1992 Oct;90(4):1296–1301. doi: 10.1172/JCI115994. [DOI] [PMC free article] [PubMed] [Google Scholar]
  32. Meyer K. C., Lewandoski J. R., Zimmerman J. J., Nunley D., Calhoun W. J., Dopico G. A. Human neutrophil elastase and elastase/alpha 1-antiprotease complex in cystic fibrosis. Comparison with interstitial lung disease and evaluation of the effect of intravenously administered antibiotic therapy. Am Rev Respir Dis. 1991 Sep;144(3 Pt 1):580–585. doi: 10.1164/ajrccm/144.3_Pt_1.580. [DOI] [PubMed] [Google Scholar]
  33. Nadel J. A. Protease actions on airway secretions. Relevance to cystic fibrosis. Ann N Y Acad Sci. 1991;624:286–296. doi: 10.1111/j.1749-6632.1991.tb17027.x. [DOI] [PubMed] [Google Scholar]
  34. Nakamura H., Yoshimura K., McElvaney N. G., Crystal R. G. Neutrophil elastase in respiratory epithelial lining fluid of individuals with cystic fibrosis induces interleukin-8 gene expression in a human bronchial epithelial cell line. J Clin Invest. 1992 May;89(5):1478–1484. doi: 10.1172/JCI115738. [DOI] [PMC free article] [PubMed] [Google Scholar]
  35. O'Connor C. M., Gaffney K., Keane J., Southey A., Byrne N., O'Mahoney S., Fitzgerald M. X. alpha 1-Proteinase inhibitor, elastase activity, and lung disease severity in cystic fibrosis. Am Rev Respir Dis. 1993 Dec;148(6 Pt 1):1665–1670. doi: 10.1164/ajrccm/148.6_Pt_1.1665. [DOI] [PubMed] [Google Scholar]
  36. Power C., O'Connor C. M., MacFarlane D., O'Mahoney S., Gaffney K., Hayes J., FitzGerald M. X. Neutrophil collagenase in sputum from patients with cystic fibrosis. Am J Respir Crit Care Med. 1994 Sep;150(3):818–822. doi: 10.1164/ajrccm.150.3.8087357. [DOI] [PubMed] [Google Scholar]
  37. Sakata K., Yajima H., Tanaka K., Sakamoto Y., Yamamoto K., Yoshida A., Dohi Y. Erythromycin inhibits the production of elastase by Pseudomonas aeruginosa without affecting its proliferation in vitro. Am Rev Respir Dis. 1993 Oct;148(4 Pt 1):1061–1065. doi: 10.1164/ajrccm/148.4_Pt_1.1061. [DOI] [PubMed] [Google Scholar]
  38. Schuster A., Fahy J. V., Ueki I., Nadel J. A. Cystic fibrosis sputum induces a secretory response from airway gland serous cells that can be prevented by neutrophil protease inhibitors. Eur Respir J. 1995 Jan;8(1):10–14. doi: 10.1183/09031936.95.08010010. [DOI] [PubMed] [Google Scholar]
  39. Shah P. L., Scott S. F., Knight R. A., Hodson M. E. The effects of recombinant human DNase on neutrophil elastase activity and interleukin-8 levels in the sputum of patients with cystic fibrosis. Eur Respir J. 1996 Mar;9(3):531–534. doi: 10.1183/09031936.96.09030531. [DOI] [PubMed] [Google Scholar]
  40. Simmons J., Chakko S., Willens H., Kessler K. M. Cost-effectiveness in clinical cardiology. II. Preventive strategles and arrhythmla therapies. Chest. 1996 Jul;110(1):256–262. doi: 10.1378/chest.110.1.256. [DOI] [PubMed] [Google Scholar]
  41. Sponer M., Nick H. P., Schnebli H. P. Different susceptibility of elastase inhibitors to inactivation by proteinases from Staphylococcus aureus and Pseudomonas aeruginosa. Biol Chem Hoppe Seyler. 1991 Nov;372(11):963–970. doi: 10.1515/bchm3.1991.372.2.963. [DOI] [PubMed] [Google Scholar]
  42. Stolk J., Camps J., Feitsma H. I., Hermans J., Dijkman J. H., Pauwels E. K. Pulmonary deposition and disappearance of aerosolised secretory leucocyte protease inhibitor. Thorax. 1995 Jun;50(6):645–650. doi: 10.1136/thx.50.6.645. [DOI] [PMC free article] [PubMed] [Google Scholar]
  43. Stone P. J., Konstan M. W., Berger M., Dorkin H. L., Franzblau C., Snider G. L. Elastin and collagen degradation products in urine of patients with cystic fibrosis. Am J Respir Crit Care Med. 1995 Jul;152(1):157–162. doi: 10.1164/ajrccm.152.1.7599816. [DOI] [PubMed] [Google Scholar]
  44. Storey D. G., Ujack E. E., Mitchell I., Rabin H. R. Positive correlation of algD transcription to lasB and lasA transcription by populations of Pseudomonas aeruginosa in the lungs of patients with cystic fibrosis. Infect Immun. 1997 Oct;65(10):4061–4067. doi: 10.1128/iai.65.10.4061-4067.1997. [DOI] [PMC free article] [PubMed] [Google Scholar]
  45. Suter S., Schaad U. B., Morgenthaler J. J., Chevallier I., Schnebli H. P. Fibronectin-cleaving activity in bronchial secretions of patients with cystic fibrosis. J Infect Dis. 1988 Jul;158(1):89–100. doi: 10.1093/infdis/158.1.89. [DOI] [PubMed] [Google Scholar]
  46. Tomee J. F., Koëter G. H., Hiemstra P. S., Kauffman H. F. Secretory leukoprotease inhibitor: a native antimicrobial protein presenting a new therapeutic option? Thorax. 1998 Feb;53(2):114–116. doi: 10.1136/thx.53.2.114. [DOI] [PMC free article] [PubMed] [Google Scholar]
  47. Vogelmeier C., Döring G. Neutrophil proteinases and rhDNase therapy in cystic fibrosis. Eur Respir J. 1996 Nov;9(11):2193–2195. doi: 10.1183/09031936.96.09112193. [DOI] [PubMed] [Google Scholar]
  48. Vogelmeier C., Hubbard R. C., Fells G. A., Schnebli H. P., Thompson R. C., Fritz H., Crystal R. G. Anti-neutrophil elastase defense of the normal human respiratory epithelial surface provided by the secretory leukoprotease inhibitor. J Clin Invest. 1991 Feb;87(2):482–488. doi: 10.1172/JCI115021. [DOI] [PMC free article] [PubMed] [Google Scholar]
  49. Vogelmeier C., Kirlath I., Warrington S., Banik N., Ulbrich E., Du Bois R. M. The intrapulmonary half-life and safety of aerosolized alpha1-protease inhibitor in normal volunteers. Am J Respir Crit Care Med. 1997 Feb;155(2):536–541. doi: 10.1164/ajrccm.155.2.9032191. [DOI] [PubMed] [Google Scholar]
  50. Wewers M. D., Casolaro M. A., Sellers S. E., Swayze S. C., McPhaul K. M., Wittes J. T., Crystal R. G. Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema. N Engl J Med. 1987 Apr 23;316(17):1055–1062. doi: 10.1056/NEJM198704233161704. [DOI] [PubMed] [Google Scholar]
  51. Witko-Sarsat V., Allen R. C., Paulais M., Nguyen A. T., Bessou G., Lenoir G., Descamps-Latscha B. Disturbed myeloperoxidase-dependent activity of neutrophils in cystic fibrosis homozygotes and heterozygotes, and its correction by amiloride. J Immunol. 1996 Sep 15;157(6):2728–2735. [PubMed] [Google Scholar]
  52. Wright G., Carver A., Cottom D., Reeves D., Scott A., Simons P., Wilmut I., Garner I., Colman A. High level expression of active human alpha-1-antitrypsin in the milk of transgenic sheep. Biotechnology (N Y) 1991 Sep;9(9):830–834. doi: 10.1038/nbt0991-830. [DOI] [PubMed] [Google Scholar]

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