Novel short chain fatty acids restore chloride secretion in cystic fibrosis

Biochemical and Biophysical Research Communications

Volumn 342, Issue 1, 2006, Pages 245-252

  Nguyen, Toan D ^a   Kim, Ug Sung ^a   Perrine, Susan P ^b  

^a UNIVERSITY OF WASHINGTON SCHOOL OF MEDICINE   (United States)

^b BOSTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

F508 CFTR; methylhydrocinnamic acid; 2,2 dimethyl butyrate; Butyrate derivatives; Chloride channel; Chloride conductance; Cystic fibrosis; IB3 1 airway cells; Intracellular trafficking; Iodide efflux

Indexed keywords

2 DIMETHYL 2 PHENYLACETIC ACID; 2,2 DIMETHYLBUTYRATE; 4 PHENYLBUTYRIC ACID; BUTYRIC ACID DERIVATIVE; CHLORIDE; CHLORIDE CHANNEL; FATTY ACID DERIVATIVE; FORSKOLIN; GLIBENCLAMIDE; HYDROCINNAMIC ACID DERIVATIVE; IODINE 125; SHORT CHAIN FATTY ACID; ST 20; ST 7; TRANSMEMBRANE CONDUCTANCE REGULATOR; UNCLASSIFIED DRUG;

ARTICLE; CELL LINE; CELL MEMBRANE; CHLORIDE CONDUCTANCE; CONTROLLED STUDY; CYSTIC FIBROSIS; DRUG BLOOD LEVEL; EPITHELIUM CELL; GENE DELETION; GENE MUTATION; HUMAN; HUMAN CELL; PRIORITY JOURNAL; PROTEIN FOLDING;

CELL LINE; CHLORIDES; CYSTIC FIBROSIS; FATTY ACIDS, VOLATILE; FORSKOLIN; HUMANS; IODINE; MOLECULAR STRUCTURE;

EID: 32644456906     PISSN: 0006291X     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.bbrc.2006.01.127     Document Type: Article

References (22)

1. G.M. Roomans Pharmacological Approaches to correcting the ion transport defect in cystic fibrosis Am. J. Respir. Med. 2 2003 413 431
2. R.C. Rubenstein, M.E. Egan, and P.L. Zeitlin In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium4-phenylbutyrate in cystic fibrosis epithelial cells containing ΔF508-CFTR J. Clin. Invest. 100 1997 2457 2465
3. S.H. Cheng, S.L. Fang, J. Zabner, J. Marshall, S. Piraino, S.C. Schiavi, D.M. Jefferson, M.J. Welsh, and A.E. Smith Functional activation of the cystic fibrosis trafficking mutant deltaF508-CFTR by overexpression Am. J. Physiol. 268 1995 L615 L624
4. R.C. Rubenstein, and P.L. Zeitlin A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in ΔF508-homozygous cystic fibrosis patients Am. J. Respir. Crit. Care Med. 157 1998 484 490
5. P.L. Zeitlin, M. Diener-West, R.C. Rubenstein, M.P. Boyle, C.K.K. Lee, and L. Brass-Ernst Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate Mol. Ther. 6 2002 119 126
6. S.P. Perrine, G.D. Ginder, D.V. Faller, G.H. Dover, T. Ikuta, H.E. Witkowska, S.-P. Cai, E.P. Vichinsky, and N.F. Oliveieri A short-term trial of butyrate to stimulate fetal-globin-gene expression in the β-globin disorders N. Engl. J. Med. 328 1993 81 86
7. B.S. Pace, G.L. White, G.J. Dover, M.S. Boosalis, D.V. Faller, and S.F. Perrine Short-chain fatty acid derivatives induce fetal globin expression and erythropoiesis in vivo Blood 100 2002 4640 4648
8. P.L. Zeitlin, L. Lu, J. Rhim, G. Cutting, G. Stetten, K.A. Kieffer, R. Craig, and W.B. Guggino A cystic fibrosis bronchial epithelial cell line: Immortalization by adeno-12-SV40 infection Am. J. Respir. Cell Mol. Biol. 4 1991 313 319
9. C.J. Venglarik, R.J. Bridges, and R.A. Frizzell A simple assay for agonist-regulated Cl and K conductances in salt-secreting epithelial cells Am. J. Physiol. 259 1990 C358 C364
10. M. Wilschanski, C. Famini, H. Blau, J. Rivlin, A. Augarten, A. Avital, B. Kerem, and E. Kerem A pilot study of the effect of gentamycin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations Am. J. Resp. Crit. Care Med. 161 2000 860 865
11. D.M. Bedwell, A. Kaenjak, D. Benos, Z. Bebok, J.K. Bubien, J. Hong, A. Tousson, J.P. Clancy, and E.J. Sorscher Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line Nat. Med. 3 1997 1280 1284
12. G.F. Atweh, M. Sutton, I. Nassif, V. Boosalis, G.J. Dover, S. Wallenstein, E. Wright, L. McMahon, G. Stamatayannopoulos, D.V. Faller, and S.P. Perrine Sustained induction of fetal hemoglobin by pulse butyrate therapy in sickle cell anemia Blood 93 1999 1790 1797
13. M. Chillon, T. Casals, B. Mercier, L. Bassas, W. Lissens, S. Silber, M.-C. Romey, J. Ruiz-Romero, C. Verlingue, M. Claustres, V. Nunes, C. Ferec, and X. Estivill Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens N. Engl. J. Med. 332 1995 1475 1480
14. G.J. Dover, S. Brusilow, and S. Charache Induction of fetal hemoglobulin in subjects with sickle cell anemia by oral sodium phenylbutyrate Blood 84 1994 339 3443
15. J. Gilbert, S.D. Baker, M.K. Bowling, L. Grochow, W.D. Figg, Y. Zabelina, R.C. Donehower, and M.A. Carducci A phase I dose escalation and bioavailability study of oral sodium phenylbutyrate in patients with refractory solid tumor malignancies Clin. Cancer Res. 7 2001 2292 2300
16. M.S. Boosalis, R. Bandyopadhyay, E.H. Bresnick, B.S. Pace, K. Van DeMark, B. Zhang, D.V. Faller, and S.P. Perrine Short-chain fatty acid derivatives stimulate cell proliferation and induce STAT-5 activation Blood 97 2001 3259 3267
17. M. Nissim-Rafinia, M. Aviram, S.H. Randell, L. Shushi, E. Ozeri, O. Chiba-Falek, O. Eidelman, H.B. Pollard, J.R. Yankaskas, and B. Kerem Restoration of the cystic fibrosis transmembrane conductance regulator function by splicing modulation EMBO Rep. 5 2004 1071 1077
18. E.M. Schwiebert, T. Flotte, G.R. Cutting, and W.B. Guggino Both CFTR and outwardly rectifying chloride channels contribute to cAMP-stimulated whole cell chloride currents Am. J. Physiol. 266 1994 C1464 C1477
19. W. Dalemans, P. Barbry, G. Champigny, S. Jallat, K. Dott, D. Dreyer, R.G. Crystal, A. Pavirani, J.-P. Lecocq, and M. Lazdunski Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation Nature 354 1991 526 528
20. C.M. Haws, 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 1996 C1544 C1555
21. M. Lim, K. McKenzie, A.D. Floyd, E. Kwon, and P.L. Zeitlin Modulation of ΔF508 cystic fibrosis transmembrane regulator trafficking and function with 4-phenylbutyrate and flavanoids Am. J. Respir. Cell Mol. Biol. 31 2004 351 357
22. G. Kuznetsov, and S.K. Nigam Folding of secretory and membrane proteins N. Engl. J. Med. 339 1998 1688 1695