Cystic fibrosis-associated mutations at arginine 347 alter the pore architecture of CFTR. Evidence for disruption of a salt bridge

Journal of Biological Chemistry

Volumn 274, Issue 9, 1999, Pages 5429-5435

  Cotten, Joseph F ^a   Welsh, Michael J ^a  

^a UNIVERSITY OF IOWA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE CHANNEL; TRANSMEMBRANE CONDUCTANCE REGULATOR;

AMINO ACID SEQUENCE; AMINO ACID SUBSTITUTION; ARTICLE; CELL MEMBRANE POTENTIAL; CHANNEL GATING; CHLORIDE CONDUCTANCE; CHLORIDE CURRENT; CONFORMATIONAL TRANSITION; NONHUMAN; PRIORITY JOURNAL; PROTEIN CONFORMATION; SITE DIRECTED MUTAGENESIS;

AMINO ACID SUBSTITUTION; ARGININE; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; ELECTROSTATICS; HYDROGEN-ION CONCENTRATION; MEMBRANE POTENTIALS; MUTAGENESIS, SITE-DIRECTED; PATCH-CLAMP TECHNIQUES; PHENOTYPE; PROTEIN CONFORMATION; SALTS;

EID: 0033605158     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.274.9.5429     Document Type: Article

References (37)

1. Riordan, J. R. (1993) Annu. Rev. Physiol. 55, 609-630
2. Hanrahan, J. W., Tabcharani, J. A., Becq, F., Mathews, C. J., Augustinas, O., Jensen, T. J., Chang, X. B., and Riordan, J. R. (1995) Soc. Gen. Physiol. Ser. 50, 125-137
3. Anderson, M. P., Gregory, R. J., Thompson, S., Souza, D. W., Paul, S., Mulligan, R. C., Smith, A. E., and Welsh, M. J. (1991) Science 253, 202-205
4. Bear, C. E., Duguay, F., Naismith, A. L., Kartner, N., Hanrahan, J. W., and Riordan, J. R. (1991) J. Biol. Chem. 266, 19142-19145
5. Berger, H. A., Anderson, M. P., Gregory, R. J., Thompson, S., Howard, P. W., Maurer, R. A., Mulligan, R., Smith, A. E., and Welsh, M. J. (1991) J. Clin. Invest. 88, 1422-1431
6. Kartner, N., Hanrahan, J. W., Jensen, T. J., Naismith, A. L., Sun, S., Ackerley, C. A., Reyes, E. F., Tsui, L.-C., Rommens, J. M., Bear, C. E., and Riordan, J. R. (1991) Cell 64, 681-691
7. Sheppard, D. N., Rich, D. P., Ostedgaard, L. S., Gregory, R. J., Smith, A. E., and Welsh, M. J. (1993) Nature 362, 160-164
8. Tabcharani, J. A., Rommens, J. M., Hou, Y.-X., Chang, X.-B., Tsui, L.-C., Riordan, J. R., and Hanrahan, J. W. (1993) Nature 366, 79-82
9. Akabas, M. H., Kaufmann, C., Cook, T. A., and Archdeacon, P. (1994) J. Biol. Chem. 269, 14865-14868
10. McDonough, S., Davidson, N., Lester, H. A., and McCarty, N. A. (1994) Neuron 13, 623-634
11. Cheung, M., and Akabas, M. H. (1996) Biophys. J. 70, 2688-2695
12. Mansoura, M. K., Smith, S. S., Choi, A. D., Richards, N. W., Strong, T. V., Drumm, M. L., Collins, F. S., and Dawson, D. C. (1998) Biophys. J. 74, 1320-1332
13. Dean, M., White, M. B., Amos, J., Gerrard, B., Stewart, C., Khaw, K. T., and Leppert, M. (1990) Cell 61, 863-870
14. Cremonesi, L., Ferrari, M., Belloni, E., Magnani, C., Seia, M., Ronchetto, P., Rady, M., Russo, M. P., Romeo, G., and Devoto, M. (1992) Hum. Mutat. 1, 314-319
15. Audrezet, M. P., Mercier, B., Guillermit, H., Quere, I., Verlingue, C., Rault, G., and Ferec, C. (1993) Hum. Mol. Genet. 2, 51-54
16. Tabcharani, J. A., Linsdell, P., and Hanrahan, J. W. (1997) J. Gen. Physiol. 110, 341-354
17. Linsdell, P., and Hanrahan, J. W. (1996) J. Physiol. (Lond.) 496, 687-693
18. Perutz, M. F. (1978) Science 201, 1187-1191
19. Kunkel, T. A. (1985) Proc. Natl. Acad. Sci. U. S. A. 82, 488-492
20. Anderson, M. P., and Welsh, M. J. (1992) Science 257, 1701-1704
21. Hamill, O. P., Marty, A., Neher, E., Sakmann, B., and Sigworth, F. J. (1981) Pflugers Arch. 391, 85-100
22. Sheppard, D. N., Ostedgaard, L. S., Winter, M. C., and Welsh, M. J. (1995) EMBO J. 14, 876-883
23. Carson, M. R., and Welsh, M. J. (1993) Am. J. Physiol. 265, L27-L32
24. Ishihara, H., and Welsh, M. J. (1997) Am. J. Physiol. 273, C1278-C1289
25. Root, M. J., and MacKinnon, R. (1994) Science 265, 1852-1856
26. Winter, M. C., Sheppard, D. N., Carson, M. R., and Welsh, M. J. (1994) Biophys. J. 66, 1398-1403
27. Gunderson, K. L., and Kopito, R. R. (1994) J. Biol. Chem. 269, 19349-19353
28. Li, C., Ramjeesingh, M., Wang, W., Garami, E., Hewryk, M., Lee, D., Rommens, J. M., Galley, K., and Bear, C. E. (1996) J. Biol. Chem. 271, 28463-28468
29. Lu, Z., and MacKinnon, R. (1995) Biochemistry 34, 13133-13138
30. Eigen, M., Hammes, G. G., and Kustin, K. (1960) J. Am. Chem. Soc. 82, 3482-3483
31. Läuger, P. (1991) Electogenic Ion Pumps,5 Ed., SinauerAssociates, Inc., Sunderland, MA
32. Gadsby, D. C., Rakowski, R. F., and DeWeer, P. (1993) Science 260, 100-103
33. King, S. C., Hansen, C. L., and Wilson, T. H. (1991) Biochim. Biophys. Acta 1062, 177-186
34. Sahin-Toth, M., Dunten, R. L., Gonzalez, A., and Kaback, H. R. (1992) Proc. Natl. Acad. Sci. U. S. A. 89, 10547-10551
35. Yang, J., Yu, M., Jan, Y. N., and Jan, L. Y. (1997) Proc. Natl. Acad. Sci. U. S. A. 94, 1568-1572
36. Antosiewicz, J., and McCammon, J. A. (1996) Biochemistry 35, 7819-7833
37. Hille, B. (1992) Ionic Channels in Excitable Membranes, Sinauer Associates, Inc., Sunderland, MA