Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

Nature

Volumn 347, Issue 6291, 1990, Pages 358-363

  Rich, Devra P ^a   Anderson, Matthew P ^a   Gregory, Richard J ^b   Cheng, Seng H ^b   Paul, Sucharita ^b   Jefferson, Douglas M ^d   McCann, John D ^a   Klinger, Katherine W ^c   Smith, Alan E ^b   Welsh, Michael J ^a  

^a UNIVERSITY OF IOWA   (United States)

^b GENZYME CORPORATION   (United States)

^c IG Laboratories Inc ^*  (United States)

^d TUFTS UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE CHANNEL;

AIRWAY; ARTICLE; CELL CULTURE; CYSTIC FIBROSIS; EPITHELIUM CELL; GENE EXPRESSION; HUMAN; HUMAN CELL; MEMBRANE CONDUCTANCE; PRIORITY JOURNAL;

CELL MEMBRANE PERMEABILITY; CELLS, CULTURED; CHLORIDE CHANNELS; CHLORIDES; CYCLIC AMP; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; ELECTRIC CONDUCTIVITY; EPITHELIUM; GENE EXPRESSION; HUMAN; KINETICS; MEMBRANE PROTEINS; MICROSCOPY, FLUORESCENCE; MUTATION; RESPIRATORY SYSTEM; SUPPORT, NON-U.S. GOV'T; SUPPORT, U.S. GOV'T, P.H.S.; TRANSFECTION; VACCINIA VIRUS;

EID: 0025155528     PISSN: 00280836     EISSN: None     Source Type: Journal    
DOI: 10.1038/347358a0     Document Type: Article

References (35)

1. Boat, T. F, Welsh, M. J. & Beaudet, A. L. in The Metablic Basis of Inherited Disease (eds Scriver, C. R., Beaudet, A. L, Sly, W. S. & Valle, D.) 2649-2860 (McGraw Hill, New York, 1989).
2. Quinton, P. FASEB J. 4, 2709-2717 (1990).
3. Welsh, M. J & Liedtke C.M. Nature 322, 467-470(1986)
4. Frizzell, R. A., Rechkemmer, G. & Shoemaker, R. L. Science 233, 558-560 (1986).
5. Welsh, M. J. FASEB J, 4, 2718-2725 (1990).
6. Schoumacher, R. A. et al. Nature 330, 752-754 (1987).
7. Li, M. et al. Nature 331, 358-360 (1988).
8. Rommens, J. M. et al. Science 245, 1059-1065 (1989).
9. Riordan, J. R. et al. Science 245, 1066-1073 (1989).
10. Kerem, B.-S. et al. Science 245, 1073-1080 (1989).
11. Lemna, W. K. et al. New Engl. J. Med. 322, 291-296 (1990).
12. Gregory, R. J. et al. Nature 347, 382-386 (1990).
13. Fuerst, T. R., Niles, E. G, Studier, F. W. & Moss, B. Proc. natn. Acad. Sci. U.S.A. 83, 8122-8126 (1986).
14. Elroy-Stein, O., Fuerst, T. R. & Moss, B. Proc. natn. Acad. Sci. U.S.A. 88, 6126-6130 (1989).
15. Leonard, R. J. et al. Proc. natn. Acad. Sci. U.S.a. 85, 7629-7633 (1989).
16. Jefferson, D. M. et al. Am. J Physiol, (in the press).
17. Moss B. in Virology eds Fields, B. N. et al, 685-703 (Raven, New York, 1985).
18. Illstey, N. P. & Verkman, A. S. Biochemistry 26, 1215-1219 (1987).
19. Ram, S. J. & Kirk, K. L. Proc. natn. Acad. Sci. U.S.A. 86, 10166-10170 (1989).
20. Chao, A. C., Widdicomb, J. H. & Verkman, A. S. J Membrane Biol. 113, 193-202 (1990).
21. Clancy, J. P., McCann, J. D. Li, M. & Welsh, M. J. Am. J. Physiol. 258, L25-32 (1990).
22. Li, M, McCann, J. D. & Welsh, M, J. Am. J. Physiol. 259, C295-C301 (1990).
23. Verkman, A. S., Sellers, M. C., Chao, A. C., Leung, T. & Ketcham, R. Analyt. Biochem. 178, 355-361 (1989).
24. Behm, J. K., Hagiwara, G., Lewiston, N. J., Quinton, P. M. & Wine, J. J. Pediat. Res. 22, 271-276 (1987).
25. Sato, K. & Sato, F. J. Lab. din. Med. Ill, 511-518 (1988).
26. Horn, R. & Marty, A, J. gen. Physiol. 92, 145-159 (1988).
27. Hamill, O. P., Marty, A., Neher, E., Sakmann, B. & Sigworth, F. J. Pflugers Arch. 391, 85-100 (1981).
28. Marty, A. & Neher, E. in Single-Channel Recording (eds Sakmann, B. & Neher, E.) 107-122 (Plenum Publishing Corporation, New York, 1983).
29. Mccann, J.D, Li, M. & Welsh, M.J. J. Gen. Physiol. 94, 1015-1036 (1989)
30. Cliff, W. H. & Frizzell, R. A. Ped. Pulm. Suppl. 4, 45 (1989).
31. DiStefano, A. et al. Pflugers Arch 405 (Suppl. 1), S95-S100 (1985).
32. Boucher, R. C., Stutts, M. J, Knowles, M. R., Cantley, L. & Gatzy, J. T. J. din. Invest. 78, 1245-1252 (1986).
33. Welsh, M. J. J. Membrane Biol. 88, 149-163 (1985).
34. Feigner, P. L. et al. Proc. natn. Acad. Sci. U.S.A. 84, 7413-7417 (1987).
35. Laemmli, U. K. Nature 227, 680-685 (1970).