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8. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. Rubenstein RC, Egan ME, Zeitlin PL. J Clin Invest; 1997 Nov 15; 100(10):2457-65. PubMed ID: 9366560 [Abstract] [Full Text] [Related]
9. A1 receptor antagonist 8-cyclopentyl-1,3-dipropylxanthine selectively activates chloride efflux from human epithelial and mouse fibroblast cell lines expressing the cystic fibrosis transmembrane regulator delta F508 mutation. Guay-Broder C, Jacobson KA, Barnoy S, Cabantchik ZI, Guggino WB, Zeitlin PL, Turner RJ, Vergara L, Eidelman O, Pollard HB. Biochemistry; 1995 Jul 18; 34(28):9079-87. PubMed ID: 7542476 [Abstract] [Full Text] [Related]
10. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. Rubenstein RC, Zeitlin PL. Am J Respir Crit Care Med; 1998 Feb 18; 157(2):484-90. PubMed ID: 9476862 [Abstract] [Full Text] [Related]
11. Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein. Illek B, Zhang L, Lewis NC, Moss RB, Dong JY, Fischer H. Am J Physiol; 1999 Oct 18; 277(4):C833-9. PubMed ID: 10516113 [Abstract] [Full Text] [Related]
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13. Correction of G551D-CFTR transport defect in epithelial monolayers by genistein but not by CPX or MPB-07. Zegarra-Moran O, Romio L, Folli C, Caci E, Becq F, Vierfond JM, Mettey Y, Cabrini G, Fanen P, Galietta LJ. Br J Pharmacol; 2002 Oct 18; 137(4):504-12. PubMed ID: 12359632 [Abstract] [Full Text] [Related]