Genetic disorders of membrane transport. II. Regulation of CFTR by small molecules including HCO3/-

American Journal of Physiology - Gastrointestinal and Liver Physiology

Volumn 275, Issue 6 38-6, 1998, Pages

  Illek, Beate ^a,b   Fischer, Horst ^a   Machen, Terry E ^b  

^a CHILDREN S HOSPITAL OAKLAND RESEARCH INSTITUTE   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Chloride secretion; Cystic fibrosis; Cystic fibrosis transmembrane conductance regulator; Epithelial transport; Pharmacology

Indexed keywords

1 (5 CHLORO 2 HYDROXYPHENYL) 5 TRIFLUOROMETHYL 2 BENZIMIDAZOLONE; 8 CYCLOPENTYL 1,3 DIPROPYLXANTHINE; BICARBONATE; CHLORIDE; GENISTEIN; ISOBUTYLMETHYLXANTHINE; MILRINONE; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ANION EXCHANGE; ARTICLE; CELL PH; CHLORIDE CURRENT; CHLORIDE TRANSPORT; CYSTIC FIBROSIS; GENETIC DISORDER; HUMAN; INTESTINE SECRETION; MEMBRANE PERMEABILITY; MEMBRANE TRANSPORT; PRIORITY JOURNAL; PROTEIN BINDING;

EID: 0032416859     PISSN: 01931857     EISSN: None     Source Type: Journal    
DOI: 10.1152/ajpgi.1998.275.6.g1221     Document Type: Article

References (19)

1. Allen, A., G. Flemstrom, A. Garner, and E. Kivilaakso. Gastroduodenal mucosal protection. Physiol. Rev. 73: 823-857, 1993.
2. Arispe, N., J. Ma, K. A. Jacobson, and H. B. Pollard. Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-diallyl-8-cyclohexylxanthine (DAX). J. Biol. Chem. 273: 5727-5734, 1998.
3. - channel activity compensates for defective processing in a cystic fibrosis mutant corresponding to a mild form of the disease. EMBO J. 14: 2417-2423, 1995.
4. Chappe, V., Y. Mettey, J. M. Vierfond, J. W. Hanrahan, M. Gola, B. Verrier, and F. Becq. Structural basis for specificity and potency of xanthine derivatives as activators of the CFTR chloride channel. Br. J. Pharmacol. 123: 683-693, 1998.
5. - secretion across murine duodenum. Am. J. Physiol. 274 (Gastrointest. Liver Physiol. 37): G718-G726, 1998.
6. Cohen, B. E., G. Lee, K. A. Jacobson, Y. C. Kim, Z. Huang, E. J. Sorscher, and H. B. Pollard. 8-cyclopentyl-1,3-dipropylxanthine and other xanthines differentially bind to the wild-type and ΔF508 first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator. Biochemistry 36: 6455-6461, 1997.
7. Crawford, I., P. C. Maloney, P. L. Zeitlin, W. B. Guggino, S. C. Hyde, H. Turley, K. C. Gatter, A. Harris, and C. F. Higgins. Immunocytochemical localization of the cystic fibrosis gene product CFTR. Proc. Natl. Acad. Sci. USA 88: 9262-9266, 1991.
8. 1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells. Proc. Natl. Acad. Sci. USA 89: 5562-5566, 1992.
9. Fischer, H., and B. Illek. Effects of genistein on disease-causing mutant CFTRs (Abstract). Pediatr. Pulmonol. Suppl 17: 239, 1998.
10. - channels via a tyrosine kinase- and protein phosphatase-independent mechanism. Am. J. Physiol. 273 (Cell Physiol. 42): C747-C753, 1997.
11. Gadsby, D. C., G. Nagel, and T. C. Hwang. The CFTR chloride channel of mammalian heart. Annu. Rev. Physiol. 57: 387-416, 1995.
12. Gribkoff, V. K., G. Champigny, P. Barbry, S. I. Dworetzky, N. A. Meanwell, and M. Lazdunski. The substituted benzimidazolono NS-004 is an opener of the cystic fibrosis chloride channel. J. Biol. Chem. 269: 10983-10986, 1994.
13. Grubb, B. R., and S. E. Gabriel. Intestinal physiology and pathology in gene-targeted mouse models of cystic fibrosis. Am. J. Physiol. 273 (Gastrointest. Liver Physiol. 36): G258-G266, 1997.
14. Grubman, S. A., R. D. Perrone, D. W. Lee, M. D. Brett, C. Jiang, S. H. Cheng, and D. M. Jefferson. Regulation of chloride/bicarbonate exchange activity by wild-type and mutant CFTR. Pediatr. Pulmonol. Suppl. 14: 277, 1997.
15. Haws, C. M., I. B. Nepomuceno, M. E. Krouse, H. Wakelee, T. Law, Y. Xia, H. Nguyen, and J. J. Wine. ΔF508-CFTR channels: kinetics, activation by forskolin, and potentiation by xanthines. Am. J. Physiol. 270 (Cell Physiol. 39): C1544-C1555, 1996.
16. Hogan, D. L., D. L. Crombie, J. I. Isenberg, P. Svendsen, O. B. Schaffalitzky de Muckadell, and M. A. Ainsworth. Acid-stimulated duodenal bicarbonate secretion involves a CFTR-mediated transport pathway in mice. Gastroenterology 113: 533-541, 1997.
17. - secretion. Am. J. Physiol. 272 (Gastrointest. Liver Physiol. 35): G872-G878, 1997.
18. Hwang, T. C., F. Wang, I. C. Yang, and W. W. Reenstra. Genistein potentiates wild-type and ΔF508-CFTR channel activity. Am. J. Physiol. 273 (Cell Physiol. 42): C988-C998, 1997.
19. - conductance of human airway epithelium in vitro and in vivo. Am. J. Physiol. 275 (Lung Cell. Mol. Physiol. 19): L902-L910, 1998.