Activation of ΔF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX

European Respiratory Journal

Volumn 15, Issue 5, 2000, Pages 937-941

  Andersson, C ^a   Roomans, G M ^a  

^a UPPSALA UNIVERSITY   (Sweden)

Author keywords

F508 cystic fibrosis transmembrane; 4 phenylbutyrate; Chloride transport; Conductance regulator; Cystic fibrosis; Genistein; X ray microanalysis

Indexed keywords

8 CYCLOPENTYL 1,3 DIPROPYLXANTHINE; ARYLBUTYRIC ACID DERIVATIVE; CYCLIC AMP DEPENDENT PROTEIN KINASE; GENISTEIN; TRANSMEMBRANE CONDUCTANCE REGULATOR; 1,3-DIPROPYL-8-CYCLOPENTYLXANTHINE; 4 PHENYLBUTYRIC ACID; 4-PHENYLBUTYRIC ACID; CFTR PROTEIN, HUMAN; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR DELTA F508; XANTHINE DERIVATIVE;

ARTICLE; CELL SURFACE; CHANNEL GATING; CHLORIDE TRANSPORT; CONTROLLED STUDY; CYSTIC FIBROSIS; GENE ACTIVATION; GENE MUTATION; HUMAN; HUMAN CELL; PRIORITY JOURNAL; RESPIRATORY EPITHELIUM; X RAY MICROANALYSIS; CELL LINE; DRUG EFFECT; EPITHELIUM CELL; GENETICS; MUTATION;

CELL LINE; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPITHELIAL CELLS; GENISTEIN; HUMANS; MUTATION; PHENYLBUTYRATES; XANTHINES;

EID: 0033625415     PISSN: 09031936     EISSN: None     Source Type: Journal    
DOI: 10.1034/j.1399-3003.2000.15e21.x     Document Type: Article

References (25)

1. Brown CR, Hong-Brown LQ, Biwersi J, Verkman AS, Welch WJ. Chemical chaperones correct the mutant phenotype of the ΔF508 cystic fibrosis transmembrane conductance regulator protein. Cell Stress Chaperones 1996; 1: 117-125.
2. Find S, Riordan JR, Williams DB. Participation of the endoplasmic reticulum chaperone calnexin (p88, 1P90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator. J Biol Chem 1994; 269: 12784-12788.
3. Cheng SH, Fang SL, Zabner J, et al. Functional activation of the cystic fibrosis trafficking mutant ΔF508-CFTR by overexpression. Am J Physiol 1995; 268: L615-L624.
4. Garcia-Bermejo L, Vilaboa NE, Perez C, Galan A, De Blas E, Aller P. Modulation of heatshock protein 70 (HSP70) gene expression by sodium butyrate in U-937 promonocytic cells: relationships with differentiation and apoptosis. Exp Cell Res 1997; 236: 268-274.
5. Rubenstein RC, Egan ME, Zeitlin PL. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing ΔF508-CFTR. J Clin Invest 1997; 100: 2457-2465.
6. Rubenstein RC, Zeitlin PL. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in ΔF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. Am J Respir Crit Care Med 1998; 157: 484-490.
7. - channels via a tyrosine kinase- and protein phosphatase-independent mechanism. Am J Physiol 1997; 273: C747-C753.
8. Hwang TC, Wang F, Yang IC, Reenstra WW. Genistein potentiates wild-type and ΔF508-CFTR channel acti-vity. Am J Physiol 1997; 273: C988-C998.
9. Wang F, Zeltwanger S, Yang IC, Nairn AC, Hwang TC. Actions of genistein on cystic fibrosis transmembrane conductance regulator channel gating. Evidence for two binding sites with opposite effects. J Gen Physiol 1998; 111: 477-490.
10. Weinreich F, Wood PG, Riordan JR, Nagel G. Direct action of genistein on CFTR. Pflugers Arch 1997; 434: 484-491.
11. Cohen BE, Lee G, Jacobson KA, et al. 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 1997; 36: 6455-6461.
12. Roomans GM. Quantitative X-ray microanalysis of biological specimens. J Electron Microsc Tech 1988; 9: 19-43.
13. 1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells. Proc Natl Acad Sci USA 1992; 89: 5562-5566.
14. Kunzelmann K, Briel M, Schreiber R, Ricken S, Nitschke R, Greger R. No evidence for direct activation of the cystic fibrosis transmembrane conductance regulator by 8-cyclopentyl-1,3-dipropylxanthine. Cell Physiol Biochem 1998; 8: 185-193.
15. 1-adenosine-receptor antagonist, regulates intracellular pH in cystic fibrosis cells. Am J Physiol 1995; 269: C226-C233.
16. Sagstrom S, Roomans GM, Wroblewski R, Keulemans JL, Hoogeveen AT, Bijman J. X-ray micro-analysis of cultured respiratory epithelial cells from patients with cystic fibrosis. Acta Physiol Scand 1992; 146: 213-220.
17. Zhang AL, Roomans GM. Multiple intracellular pathways for regulation of chloride secretion in cultured pig tracheal submucosal gland cells. Eur Respir J 1999; 13: 571-576.
18. Mork AC, Hongpaisan J, Roomans GM. Ion transport in primary cultures from human sweat gland coils studied with X-ray microanalysis. Cell Biol Int 1995; 19: 151-159.
19. Hongpaisan J, Roomans GM. Regulation of ion content in primary cultures from reabsorptive ducts of human sweat glands studied by X-ray microanalysis. Cell Struct Funct 1998; 23: 239-245.
20. Reenstra WW, Yurko-Mauro K, Dam A, Raman S, Shorten S. CFTR chloride channel activation by genistein: the role of serine/threonine protein phosphatases. Am J Physiol 1996; 271: C650-C657.
21. Illek B, Fischer H, Machen TE. Alternate stimulation of apical CFTR by genistein in epithelia. Am J Physiol 1996; 270: C265-C275.
22. Yang IC, Cheng TH, Wang F, Price EM, Hwang TC. Modulation of CFTR chloride channels by calyculin A and genistein. Am J Physiol 1997; 272: C142-C155.
23. Barnes S, Sfakianos J, Coward L, Kirk M. Soy isoflavonoids and cancer prevention. Underlying biochemical and pharmacological issues. Adv Exp Med Biol 1996; 401: 87-100.
24. Lian F, Li Y, Bhuiyan M, Sarkar FH. pS3-independent apoptosis induced by genistein in lung cancer cells. Nutr Cancer 1999; 33: 125-131.
25. Anthony MS, Clarkson TB, Williams JK. Effects of soy isoflavones on atherosclerosis: potential mechanisms. Am J Clin Nutr 1998; 68: 1390S-1393S.