Genetics and cell biology in cystic fibrosis;Génétique et biologie cellulaire de la mucoviscidose

Revue du Praticien

Volumn 53, Issue 2, 2003, Pages 130-134

  Chinet, Thierry ^a   Blouquit, Sabine ^a  

^a NONE

Author keywords

[No Author keywords available]

Indexed keywords

CHLORINE; PHENYLALANINE; SODIUM; TRANSMEMBRANE CONDUCTANCE REGULATOR;

AMINO ACID SEQUENCE; ARTICLE; AUTOSOMAL RECESSIVE INHERITANCE; CAUCASIAN; CHLORIDE TRANSPORT; CYSTIC FIBROSIS; CYTOLOGY; DEHYDRATION; GENE FUNCTION; GENE MUTATION; GENETIC ANALYSIS; GENETIC CODE; HUMAN; IMMUNE RESPONSE; LUNG INFECTION; MEMBRANE TRANSPORT; MUTATIONAL ANALYSIS; PATHOPHYSIOLOGY; SODIUM TRANSPORT; SWEAT TEST;

EID: 0037439192     PISSN: 00352640     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (22)

1. Davis PB, Drumm M, Konstan MW. Cystic Fibrosis. State of the Art. Am J Resp Crit Care Med 1996; 154: 1229-56.
2. Sheppard DN, Welsh MJ. Structure and function of the CFTR chloride channel. Physiol Rev 1999; 79: S23-S45.
3. Kunzelmann K, Schreiber R. CFTR, a regulator of channels. J Membrane Biol 1999; 168: 1-8.
4. Chinet Th. Physiopathologie de la sécrétion épithéliale. In: Navarro J, Bellon G (eds). La Mucoviscidose. Montpellier: Ed Espaces 34, 2001: 33-48.
5. Chinet Th. Mucoviscidose. In: Friedlander G, Clérici C (eds). Biologie et pharmacologie des épithéliums. Paris: EDK, 2000: 89-96.
6. Pilewski JM, Frizzell RA. Role of CFTR in airway disease. Physiol Rev 1999; 79: S215-S255.
7. 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.
8. Costes B, Girodon-Boulandet E, Pagesy P, Fanen P, Goossens M. La génétique. In: Navarro J, BelIon G (eds). La Mucoviscidose. Montpellier: Espaces 34, 2001: 16-32.
9. Rozmahel, R, Wilschanki M, Matin A et al. Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor. Nature Genet 1996; 12: 280-7.
10. Boucher RC. Human airway ion transport. Am J Respir Crit Care Med 1994; 150: 271-81 et 581-93.
11. Boucher RC. Molecular insights into the physiology of the "thin film" of airway surface liquid. J Physiol (London) 1999; 516: 631-8.
12. De Bentzmann S, Roger P, Puchelle E. Pseudomonas aeruginosa adherence to remodelling respiratory epithelium. Eur Respir J 1996; 9: 2145-50.
13. Plotkowski MC, Debentzmann S, Pereira SHM et al. Pseudomonas aeruginosa internalization by human epithelial respiratory cells depends on cell differentiation, polarity, and junctional complex integrity. Am J Respir Cell Mol Biol 1999; 20: 880-90.
14. 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.
15. 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.
16. Hendry J, Elborn JS, Nixon L, Shale DJ, Webb AK. Cystic fibrosis: inflammatory response to infection with Burkholderia cepacia and Pseudomonas aeruginosa. Eur Respir J 1999; 14: 435-38.
17. Denning GM, Wollenweber LA, Railsback MA, Cox CD, Stol LL, Britigan BE. Pseudomonas pyocyanin increases interleukin-8 expression by human airway epithelial cells. Infect Immun 1998; 66: 5777-84.
18. Britigan BE, Railsback MA, Cox CD. The Pseudomonas aeruginosa secretory product pyocyanin inactivates alpha(I) protease inhibitor: Implications for the pathogenesis of cystic fibrosis lung disease. Infect lmmun 1999; 67: 1207-12.
19. 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.
20. Tabary O, Zahm J, Hinnrasky J et al. Genistein inhibits constitutive and inducible NF?B activation and decreases IL-8 production by human cystic fibrosis bronchial gland cells. Am J Pathol 1999; 155: 473-81.
21. Bonfield T, Konstan M, Burfeind P et al. Normal bronchial epithelial cells constitutively produce the anti-inflammatory interleukin-10, which is downregulated in cystic fibrosis. Am J Respir Cell Mol Biol 1995; 13: 257-61.
22. Quinton PM. Physiological basis of cystic fibrosis: a historical perspective. Physiol Rev 1999; 79: S3-S22.