CFTR and transepithelial ionic transport abnormalities in CF;CFTR et les anomalies des transports ioniques dans la mucoviscidose

Archives de Pediatrie

Volumn 10, Issue SUPPL. 2, 2003, Pages

  Becq, F ^a  

^a UNIVERSITÉ DE POITIERS   (France)

Author keywords

CFTR; Cystic fibrosis; Epithelial ion transport

Indexed keywords

CHLORIDE CHANNEL; TRANSMEMBRANE CONDUCTANCE REGULATOR;

CHANNEL GATING; CHLORIDE TRANSPORT; CONFERENCE PAPER; CYSTIC FIBROSIS; GENE MUTATION; HUMAN; ION TRANSPORT; LUNG ALVEOLUS EPITHELIUM; LUNG CLEARANCE; MEMBRANE TRANSPORT; MOLECULAR CLONING; MUCOCILIARY CLEARANCE; PATHOPHYSIOLOGY; PROTEIN FUNCTION; REGULATORY MECHANISM;

EID: 0042876790     PISSN: 0929693X     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0929-693X(03)90630-0     Document Type: Conference Paper

References (19)

1. Riordan JR, Rommens JM, Kerem BS, Alon R, Rozmahel R, Grzelczak Z, et al. Identification of the cystic fibrosis gene. Cloning and characterization of complementary DNA. Science 1989; 245: 1066-73.
2. Quinton PM. Physiological basis of cystic fibrosis: a historical perspective. Physiol Rev 1999; 79: S3-S22.
3. Davis PB, Drumm M, Konstan MW. Cystic Fibrosis. State of the art. Am J Respi Crit Care Med 1996; 154: 1229-56.
4. Phillipson G. Cystic fibrosis and reproduction. Reprod Fertil Dev 1998; 10: 113-9.
5. Riordan JR. The cystic fibrosis transmembrane conductance regulator. Annu Rev Physiol 1993; 55: 609-30.
6. Higgins CF. The ABC of channel regulation. Cell 1995; 82: 693-6.
7. - channel in CHO cells stably expressing the cystic fibrosis gene. Nature 1991; 352: 628-31.
8. Sheppard DN, Welsh MJ. Structure and function of the CFTR chloride channel. Physiol Rev 1999; 79: S23-S45.
9. Gadsby DC, Nairn AC. Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis. Physiol Rev 1999; 79: S77-S107.
10. Gray MA, Winpenny JP, Verdon B, McAlroy H, Argent BE. Chloride channels and cystic fibrosis of the pancreas. Biosc Rep 1995; 15: 531-41.
11. Wilschanski M, Durie PR, Pathology of pancreatic and intestinal disorders in cystic fibrosis. J R Soc Med 1998; 91 suppl 34: 40-9.
12. Kopito RR. Biosynthesis and degradation of CFTR. Physiol Rev 1999; 79: S167-S173.
13. Di Sant-Agnese PA, Darling RC, Perera GA, Shea E. Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas. Pediatrics 1953; 12: 549-63.
14. Schwiebert EM, Benos DJ, Egan ME, Stutts MJ, Guggino WB. CFTR is a conductance regulator as well as a chloride channel. Physiol Rev 1999; 79: S145-S166.
15. Matsui H, Grubb BR, Tarran R, Randell SH, Gatzy JT, Davis CW, et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airway disease. Cell 1998; 95: 1005-15.
16. Smith JJ, Travis SM, Greenberg EP, Welsh MJ. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell 1996; 85: 229-36.
17. Welsh MJ, Smith AE. Molecular mechanisms of CFTR chloride channel dysfunction in CF. Cell 1993; 73: 1251-4.
18. Zeitlin PL. Novel pharmacologic therapies for cystic fibrosis. J Clin Invest 1999; 103: 447-52.
19. Zielenski J, Tsui L-C. Cystic fibrosis: genotype and phenotypic variations. Annu Rev Genet 1995; 29: 777-807.