Organic Solutes Rescue the Functional Defect in ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator

Journal of Biological Chemistry

Volumn 278, Issue 51, 2003, Pages 51232-51242

  Zhang, Xue Mei ^a   Wang, Xi Tao ^a   Yue, Hongwen ^a   Leung, Steve W ^a   Thibodeau, Patrick H ^b   Thomas, Philip J ^b   Guggino, Sandra E ^a  

^a JOHNS HOPKINS UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b UNIVERSITY OF TEXAS SOUTHWESTERN MEDICAL CENTER   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AGGLOMERATION; CELLS; FLUORESCENCE; LOW TEMPERATURE EFFECTS; PROTEINS;

PLASMA MEMBRANES; TRANSMEMBRANE;

BIOCHEMISTRY;

CHLORIDE CHANNEL; FORSKOLIN; GREEN FLUORESCENT PROTEIN; INOSITOL; METHYLAMINE; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ARTICLE; CELL SURFACE; CYSTIC FIBROSIS; ENDOPLASMIC RETICULUM; GENE EXPRESSION REGULATION; GLYCOSYLATION; HUMAN; HUMAN CELL; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN DENATURATION; PROTEIN FOLDING; PROTEIN PROCESSING; PROTEIN STABILITY; SOLUTE;

ANIMALS; BETAINE; CELL LINE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; ELECTROPHYSIOLOGY; FRAMESHIFT MUTATION; GLYCOSYLATION; HUMANS; INOSITOL; ORGANIC CHEMICALS; PROTEIN FOLDING; PROTEIN TRANSPORT; TAURINE; TRANSFECTION;

EID: 0347065355     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.M309076200     Document Type: Article

References (59)

1. Kartner, N., Augustinas, O., Jensen, T. J., Naismith, A. L., and Riordan, J. R. (1992) Nat. Genet. 1, 321-327
2. Denning, G. M., Ostedgaard, L. S., and Welsh, M. J. (1992) J. Cell Biol. 118, 551-559
3. Cheng, S. H., Gregory, R. J., Marshall, J., Paul, S., Souza, D. W., White, G. A., O'Riordan, C. R., and Smith, A. E. (1990) Cell 63, 827-834
4. Sato, S., Ward, C. L., and Kopito, R. R. (1998) J. Biol. Chem. 273, 7189-7192
5. Dalemans, W., Barbry, P., Champigny, G., Jallat, S., Dott, K., Dreyer, D., Crystal, R. G., Pavirani, A., Lecocq, J. P., and Lazdunski, M. (1991) Nature 354, 526-528
6. Drumm, M. L., Wilkinson, D. J., Smit, L. S., Worrell, R. T., Strong, T. V., Frizzell, R. A., Dawson, D. C., and Collins, F. S. (1991) Science 254, 1797-1799
7. Denning, G. M., Anderson, M. P., Amara, J. F., Marshall, J., Smith, A. E., and Welsh, M. J. (1992) Nature 358, 761-764
8. Brown, C. R., Hong-Brown, L. Q., Biwersi, J., Verkman, A. S., and Welch, W. J. (1996) Cell Stress Chaperones 1, 117-125
9. Sato, S., Ward, C. L., Krouse, M. E., Wine, J. J., and Kopito, R. R. (1996) J. Biol. Chem. 271, 635-638
10. Taylor, L. S., York, P., Williams, A. C., Edwards, H. G., Mehta, V., Jackson, G. S., Badcoe, I. G., and Clarke, A. R. (1995) Biochim. Biophys. Acta 1253, 39-46
11. Back, J. F., Oakenfull, D., and Smith, M. B. (1979) Biochemistry 18, 5191-5196
12. Gekko, K. (1981) J. Biochem. (Tokyo) 90, 1633-1641
13. Gekko, K., and Ito, H. (1990) J. Biochem. (Tokyo) 107, 572-577
14. Wang, A., and Bolen, D. W. (1997) Biochemistry 36, 9101-9108
15. Howard, M., Fischer, H., Roux, J., Santos, B. C., Gullans, S. R., Yancey, P. H., and Welch, W. J. (2003) J. Biol. Chem. 278, 35159-35167
16. Hamosh, A., Rosenstein, B. J., and Cutting, G. R. (1992) Hum. Mol. Genet. 1, 542-544
17. Gregory, R. J., Cheng, S. H., Rich, D. P., Marshall, J., Paul, S., Hehir, K., Ostedgaard, L., Klinger, K. W., Welsh, M. J., and Smith, A. E. (1990) Nature 347, 382-386
18. Moyer, B. D., Loffing, J., Schwiebert, E. M., Loffing-Cueni, D., Halpin, P. A., Karlson, K. H., Ismailov, I. I., Guggino, W. B., Langford, G. M., and Stanton, B. A. (1998) J. Biol. Chem. 273, 21759-21768
19. Johnston, J. A., Ward, C. L., and Kopito, R. R. (1998) J. Cell Biol. 143, 1883-1898
20. Qu, B. H., and Thomas, P. J. (1996) J. Biol. Chem. 271, 7261-7264
21. Qu, B. H., Strickland, E. H., and Thomas, P. J. (1997) J. Biol. Chem. 272, 15739-15744
22. Egan, M. E., Schwiebert, E. M., and Guggino, W. B. (1995) Am. J. Physiol. 268, C243-C251
23. Schwiebert, E. M., Morales, M. M., Devidas, S., Egan, M. E., and Guggino, W. B. (1998) Proc. Natl. Acad. Sci. U. S. A. 95, 2674-2679
24. Bedwell, D. M., Kaenjak, A., Benos, D. J., Bebok, Z., Bubien, J. K., Hong, J., Tousson, A., Clancy, J. P., and Sorscher, E. J. (1997) Nat. Med. 3, 1280-1284
25. Peter, K., Varga, K., Bebok, Z., McNicholas-Bevensee, C. M., Schwiebert, L., Sorscher, E. J., Schwiebert, E. M., and Collawn, J. F. (2002) J. Biol. Chem. 277, 49952-49957
26. Egan, M., Flotte, T., Afione, S., Solow, R., Zeitlin, P. L., Carter, B. J., and Guggino, W. B. (1992) Nature 358, 581-584
27. Sheppard, D. N., and Welsh, M. J. (1999) Physiol. Rev. 79, S23-S45
28. Schwiebert, E. M., Benos, D. J., Egan, M. E., Stutts, M. J., and Guggino, W. B. (1999) Physiol. Rev. 79, S145-S166
29. Yue, H., Devidas, S., and Guggino, W. B. (2000) J. Biol. Chem. 275, 10030-10034
30. Whiteside, C. I., Thompson, J. C., and Ohayon, J. (1991) Am. J. Physiol. 260, F138-F144
31. Borden, L. A., Smith, K. E., Gustafson, E. L., Branchek, T. A., and Weinshank, R. L. (1995) J. Neurochem. 64, 977-984
32. Rasola, A., Galietta, L. J., Barone, V., Romeo, G., and Bagnasco, S. (1995) FEBS Lett. 373, 229-233
33. Huxtable, R. J. (1992) Physiol. Rev. 72, 101-163
34. Sone, M., Albrecht, G. J., Dorge, A., Thurau, K., and Beck, F. X. (1993) Am. J. Physiol. 264, F722-F729
35. Burg, M. B. (1995) Am. J. Physiol. 268, F983-F996
36. Kendler, B. S. (1989) Prev. Med. 18, 79-100
37. Gordon, R. E., Shaked, A. A., and Solano, D. F. (1986) Am. J. Pathol. 125, 585-600
38. Gordon, R. E., Heller, R. F., and Heller, R. F. (1992) Adv. Exp. Med. Biol. 315, 319-328
39. Barak, A. J., Beckenhauer, H. C., and Tuma, D. J. (1996) Alcohol 13, 395-398
40. Barak, A. J., Beckenhauer, H. C., Badakhsh, S., and Tuma, D. J. (1997) Alcohol Clin. Exp. Res. 21, 1100-1102
41. Roum, J. H., Buhl, R., McElvaney, N. G., Borok, Z., and Crystal, R. G. (1993) J. Appl. Physiol. 75, 2419-2424
42. Gao, L., Kim, K. J., Yankaskas, J. R., and Forman, H. J. (1999) Am. J. Physiol. 277, L113-L118
43. van der Vliet, A., and Cross, C. E. (2000) Curr. Opin. Pulm. Med. 6, 533-539
44. Thomas, P. J., Qu, B. H., and Pedersen, P. L. (1995) Trends Biochem. Sci. 20, 456-459
45. Dobson, C. M. (1999) Trends Biochem. Sci. 24, 329-332
46. Soto, C. (2001) FEBS Lett. 498, 204-207
47. Tamarappoo, B. K., and Verkman, A. S. (1998) J. Clin. Invest. 101, 2257-2267
48. Tamarappoo, B. K., Yang, B., and Verkman, A. S. (1999) J. Biol. Chem. 274, 34825-34831
49. Morello, J. P., Salahpour, A., Laperriere, A., Bernier, V., Arthus, M. F., Lonergan, M., Petaja-Repo, U., Angers, S., Morin, D., Bichet, D. G., and Bouvier, M. (2000) J. Clin. Invest. 105, 887-895
50. Foster, B. A., Coffey, H. A., Morin, M. J., and Rastinejad, F. (1999) Science 286, 2507-2510
51. Collins, K. D. (1997) Biophys. J. 72, 65-76
52. Wimmer, H., Olsson, M., Petersen, M. T., Hatti-Kaul, R., Peterson, S. B., and Muller, N. (1997) J. Biotechnol. 55, 85-100
53. Sharma, M., Benharouga, M., Hu, W., and Lukacs, G. L. (2001) J. Biol. Chem. 276, 8942-8950
54. Ward, C. L., and Kopito, R. R. (1994) J. Biol. Chem. 269, 25710-25718
55. Nakanishi, T., Turner, R. J., and Burg, M. B. (1989) Proc. Natl. Acad. Sci. U. S. A. 86, 6002-6006
56. Haworth, J. C., Dilling, L. A., Surtees, R. A., Seargeant, L. E., Lue-Shing, H., Cooper, B. A., and Rosenblatt, D. S. (1993) Am. J. Med. Genet. 45, 572-576
57. Holme, E., Kjellman, B., and Ronge, E. (1989) Arch. Dis. Child 64, 1061-1064
58. Abdelmalek, M. F., Angulo, P., Jorgensen, R. A., Sylvestre, P. B., and Lindor, K. D. (2001) Am. J. Gastroenterol. 96, 2711-2717
59. Darling, P. B., Lepage, G., Leroy, C., Masson, P., and Roy, C. C. (1985) Pediatr. Res. 19, 578-582