Mechanisms of cystic fibrosis lung disease;Physiopathologie de l'atteinte pulmonaire de la mucoviscidose

Revue des Maladies Respiratoires

Volumn 16, Issue 3, 1999, Pages 339-345

  Chinet, T ^a  

^a HÔPITAL AMBROISE PARÉ   (France)

Author keywords

Airway epithelium; CFTR; Cystic fibrosis; Inflammation; Ion transport; Pseudomonas aeruginosa

Indexed keywords

ANTIINFLAMMATORY AGENT; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ARTICLE; BACTERIAL INFECTION; CYSTIC FIBROSIS; DEFENSE MECHANISM; GENE MUTATION; GENETIC DISORDER; HUMAN; INFLAMMATION; ION TRANSPORT; LUNG DISEASE; MUCOCILIARY CLEARANCE; PATHOPHYSIOLOGY; RESPIRATORY EPITHELIUM; RESPIRATORY TRACT MUCOSA; WATER TRANSPORT;

EID: 0032813215     PISSN: 07618425     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (57)

1. FÉREC C, MERCIER B, AUDRÉZET MP : Les mutations de la mucoviscidose: du génotype au phénotype. Med Sci 1994;10:631-9.
2. ROMMENS JM, IANNUZZI MC, KEREM BS et al. : Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 1989;245:1059-64.
3. RIORDAN JR, ROMMENS JM, KEREM BS et al. : Identification of the cystic fibrosis bene: cloning and characterisation of complementary DNA. Science 1989;245:1066-73.
4. KEREM BS, ROMMENS JM, BUCHANAN JA et al. : Identification of the cystic fibrosis gene: genetic analysis. Science 1989;245:1073-80.
5. RIORDAN JR : The cystic fibrosis transmembrane conductance regulator. Annu Rev Physiol 1993 ;55:609-30.
6. FRIZZELL RA : Functions of the cystic fibrosis transmembrane conductance regulator protein. Am J Respir Crit Care Med 1995;151:S54-S58.
7. CHINET T : Protéine CFTR et mécanismes moléculaires de l'atteinte pulmonaire de la mucoviscidose. Rev Pneumol Clin 1995;51:122-9.
8. CRAWFORD I, MALONEY PC, ZEITLIN PL et al. : Immunocytochemical localization of the cystic fibrosis gene product CFTR. Proc Natl Acad Sci USA 1991;88:9262-6.
9. ZEITLIN PL, CRAWFORD I, LU L et al. : CFTR protein expression in primary and cultured epithelia. Proc Natl Acad Sci USA 1992;89:4-347.
10. TRAPNELL BC, CHU C-S, PAAKKO PK et al. : Expression of the cystic fibrosis transmembrane conductance regulator gene in the respiratory tract of normal individuals and individuals with cystic fibrosis. Proc Natl Acad Sci USA 1991;88:6565-9.
11. ENGELHARDT JR, YANKASKAS JR, ERNST SA et al. : Submucosal glands are the predominant site of CFTR expression in the human bronchus. Nature Genetics 1992;2:240-8.
12. TSUI LC : Mutations and sequence variations detected in the cystic fibrosis transmembrane conductance regulator (CFTR) gene : a report from the Cystic Fibrosis Genetic Analysis Consortium. Human Mutation 1992;1:197-203.
13. ZIELENSKI J, TSUI LC : Cystic fibrosis: genotypic and phenotypic variations. Annu Rev Genet 1995;29:777-807.
14. WELSH MJ, SMITH AE : Molecular mechanisms of CFTR chloride channels dysfunction in cystic fibrosis. Cell 1993;73:1251-4.
15. CHILLON M, CASALS T, MERCIER B et al. : Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens. N Eng J Med 1995;332:1475-80.
16. ROZMAHEL R, WILSCHANSKI M, MATIN A et al. : Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor. Nat Genet 1996;12:280-7.
17. BOUCHER RC : Human airway ion transport (part one). Am J Respir Crit Care Med 1994;150:271-81.
18. BOUCHER RC : Human airway ion transport (part two). Am J Respir Crit Cure Med 1994;150:581-93.
19. CHINET TH, FULLTON JM, YANKASKAS JR, BOUCHER RC, STUTTS MJ : Sodium-permeable channels in the apical membrane of human nasal epithelial cells. Am J Physiol (Cell Physiol) 1993;265:C1050-C1060.
20. WELSH MJ, ANDERSON MP, RICH DP et al. : Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation. Neuron 1992;8:821-9.
21. GABRIEL SE, CLARKE LL, BOUCHER RC, STUTTS MJ : CFTR and outward rectifying chloride channels are distinct proteins with a regulatory relationship. Nature 1993;363:263-6.
22. STUTTS MJ, ROSSIER BC, BOUCHER RC : Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics. J Biol Chem 1997;272:14037-40.
23. + currents. Am J Physiol (Cell Physiol) 1996;40:C1565-C1573.
24. SCHREIBER R, GREGER R, NITSCHKE R, KUNZELMANN K : Cystic fibrosis transmembrane conductance regulator activates water conductance in Xenopus oocytes. Pflugers Arch Eur J Physiol 1997;434:841-7.
25. BENALI R, PIERROT D, ZAHM JM, DE BENTZMANN S, PUCHELLE E : Effect of extracellular ATP and UTP on fluid transport by human nasal epithelial cells in culture. Am J Respir Cell Mol Biol 1994;10:363-8.
26. BENNETT WD, OLIVIER KN, ZEMAN KL, HOHNEKER KW, BOUCHER RC, KNOWLES MR : Effect of uridine 5′-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis patients. Am J Respir Crit Care Med 1996; 153:1796-801.
27. YAMAYA M, FINKBEINER WE, WIDDICOMBE JH : Ion transport by cultures of human tracheobronchial submucosal glands. Am J Physiol (Lung Physiol) 1991;261:L485-L490.
28. YAMAYA M, FINKBEINER WE, WIDDICOMBE JH : Altered ion transport by tracheal glands in cystic fibrosis. Am J Physiol (Lung Physiol) 1991;261:L491-L494.
29. JIANG C, FINKBEINER WE, WIDDICOMBE JH, MILLER SS : Fluid transport across cultures of human tracheal glands is altered in cystic fibrosis. J Physiol (London) 1997;501:637-47.
30. CARNOY C, RAMPHAL R, SCHARFMAN A et al. : Altered carbohydrate composition of salivary mucins from patients with cystic fibrosis and the adhesion of Pseudomonas aeruginosa. Am J Respir Cell Mol Biol 1993;9:323-34.
31. ZHANG Y, DORANZ B, YANKASKAS JR, ENGELHARDT JF : Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis. J Clin Invest 1995;96:2997-3004.
32. CHENG PW, BOAT TF, CRANFILL K, YANKASKAS JR, BOUCHER RC : Increased sulfation of glycoconjugates by cultured nasal epithelial cells from patients with cystic fibrosis. J Clin Invest 1989;84:68-72.
33. BARASH J, KISS B, PRINCE A, SAIMAN L, GRUENERT D, AL-AWQATI Q : Defective acidification of intracellular organelles in cystic fibrosis. Nature 1991;352:70-3.
34. SAJJAN U, REISMAN J, DOIG P, IRVIN RT, FORSTNER G, FORSTNER J : Binding of nonmucoid Pseudomonas aeruginosa to normal human intestinal mucin and respiratory mucin from patients with cystic fibrosis. J Clin Invest 1992;89:651-65.
35. KNOWLES MR, ROBINSON JM, WOOD RE et al : Ion composition of airway surface liquid of patients with cystic fibrosis as compared with normal and disease-control subjects. J Clin Invest 1997;100:2588-95.
36. HULL J, SKINNER W, ROBERTSON C, PHELAN P : Elemental content of airway surface liquid from infants with cystic fibrosis. Am J Respir Crit Care Med 1998;157:10-4.
37. MIZGERD JP, KOBZIK L, WARNER AE, BRAIN JD : Effects of sodium concentration on neutrophil bactericidal functions. Am J Physiol 1995;169:L388-L393.
38. JORIS L, DAB I, QUINTON PM : Elemental composition of human airway surface liquid in healthy and diseased airways. Am Rev Respir Dis 1993;148:1633-7.
39. GILLJAM H, ELLIN A, STRANDVIK B : Increased bronchial chloride concentration in cystic fibrosis. Scand J Clin Lab Invest 1989;49:121-4.
40. SMITH JJ, TRAVIS SM, GREENBERG EP, WELSH MJ : Cystic fibrosis airway epithelial fail to kill bacteria because of abnormal airway surface fluid. Cell 1996;85:229-36.
41. GOLDMAN MJ, ANDERSON GM, STOLZENBERG ED, PRASAD KARI U, ZASLOFF M, WILSON JM : Human β-defensin-1 is a salt sensitive antibiotic in lung that is inactivated in cystic fibrosis. Cell 1997;88:553-60.
42. MCCRAY PB, BENTLEY L : Human airway epithelia express a β-defensin. Am J Respir Cell Mol Biol 1997;16:343-9.
43. SOONG LB, GANZ T, ELLISON A, CAUGHEY GH : Purification and characterization of defensins from cystic fibrosis sputum. Inflamm Res 1997;46:98-102.
44. ZAHM JM, CHEVILLARD M, PUCHELLE E : Wound repair of human surface respiratory epithelium. Am J Respir Cell Mol Biol 1991;5:242-8.
45. DE BENTZMANN S, ROGER P, PUCHELLE E : Pseudomonas aeruginosa adherence to remodelling respiratory epithelium. Eur Resp J 1996;9:2145-50.
46. PIER GB, GROUT M, ZAIDI TS, OLSEN JC, JOHNSON LG, YANKASKAS JR, GOLDBERG JB : Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections. Science 1996;271:64-7.
47. PIER GB, GROUT M, ZAIDI TS : Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung. Proc Natl Acad Sci USA 1997;94:12088-93.
48. SUTER S, SCHAAD UB, TEGNER H, OHLSSON K, DESGRANDCHAMPS D, WALDVOGEL FA : Levels of free granulocyte elastase in bronchial secretions from patients with cystic fibrosis: effect of antimicrobial treatment against Pseudomonas aeruginosa. J Infect Dis 1986;153:902-9.
49. MCELVANEY NG, HUBBARD RC, BIRRER P et al. : Aerosol administration of alpha1-antitrypsin to suppress the burden of active neutrophil elastase on the respiratory epithelial surface in cystic fibrosis. Lancet 1991;337:392-4.
50. BIRRER P, MCELVANEY NG, RÜDEBERG A et al. : Protease-antiprotease imbalance in the lungs of children with cystic fibrosis. Am J Respir Crit Care Med 1994;150:207-13.
51. KHAN TZ, WAGENER JS, BOST T, MARTINEZ J, ACCURSO FJ, RICHES DWH : Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med 1995;151:1075-82.
52. ARMSTRONG DS, GRIMWOOD K, CARLIN JB et al. : Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med 1997;156:1197-204.
53. FAHY V, SCHUSTER A, UEKI I, BOUSHEY HA, NADEL JA : Mucus hypersecretion in bronchectasis: the role of neutrophil proteases. Am Rev Respir Dis 1992;146:1430-3.
54. MAIZIERES M, KAPLAN H, MILLOT JM et al. : Neutrophil elastase promotes rapid exocytosis in human airway gland cells by producing cytosolic Ca oscillations. Am J Respir Cell Mol Biol 1998;18:32-42.
55. SOMERVILLE M, RICHARDSON PS, RUTMAN A, WILSON R, COLE PJ : Stimulation of secretion into human and feline airways by Pseudomonas aeruginosa proteases. J Appl Physiol 1991;70:2259-67.
56. LI JD, DOHRMAN AF, GALLUP M et al. : Transcriptional activation of mucin by Pseudomonas aeruginosa lipopolysaccharide in the pathogenesis of cystic fibrosis lung disease. Proc Natl Acad Sci USA 1997;94:967-72.
57. OGRINC G, KAMPALATH B, TOMASHEFSKI JF : Destruction and loss of bronchial cartilage in cystic fibrosis. Hum Pathol 1998;29:65-73.