Channel-lining residues in the M3 membrane-spanning segment of the cystic fibrosis transmembrane conductance regulator

Biochemistry

Volumn 37, Issue 35, 1998, Pages 12233-12240

  Akabas, Myles H ^a  

^a Och Spine at New York Presbyterian Hospitals   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE CHANNEL; ION CHANNEL; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ANIMAL CELL; ARTICLE; CYSTIC FIBROSIS; GENE MUTATION; GENETIC DISORDER; MEMBRANE ELECTROPHYSIOLOGY; NONHUMAN; OOCYTE; PRIORITY JOURNAL; XENOPUS LAEVIS;

ANIMALS; CHLORIDE CHANNELS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; ETHYL METHANESULFONATE; GENETIC VECTORS; HUMANS; ION CHANNEL GATING; MEMBRANE PROTEINS; MESYLATES; MUTAGENESIS, SITE-DIRECTED; OOCYTES; PEPTIDE FRAGMENTS; PROTEIN STRUCTURE, SECONDARY; SEQUENCE DELETION; WATER; XENOPUS LAEVIS;

ANIMALIA; XENOPUS LAEVIS;

EID: 0032169499     PISSN: 00062960     EISSN: None     Source Type: Journal    
DOI: 10.1021/bi980969o     Document Type: Article

References (63)

1. Riordan, J. R., Rommens, J. M., Kerem, B. S., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Lok, S., Plavsic, N., Chou, J. L., Drumm, M. T., Iannuzzi, M. C., Collins, F. S., and Tsui, L. C. (1989) Science 254, 1066-1073.
2. Riordan, J. R. (1993) Annu. Rev. Physiol. 55, 609-630.
3. Gadsby, D. C., Nagel, G., and Hwang, T.-C. (1995) Annu. Rev. Physiol. 57, 387-416.
4. Egan, M., Flotte, T., Afione, S., Solow, R., Zeitlin, P. L., Carter, B. J., and Guggino, W. B. (1992) Nature 358, 581-584.
5. Gabriel, S. E., Clarke, L. C., Boucher, R. C., and Stutts, M. J. (1993) Nature 363, 263-266.
6. Schwiebert, E. M., Egan, M. E., Hwang, T. H., Fulmer, S. B., Allen, S. A., Cutting, G. R., and Guggino, W. B. (1995) Cell 81, 1063-1073.
7. Stutts, M. J., Canessa, C. M., Olsen, J. C., Hamrick, M., Cohn, J. A., Rossier, B. C., and Boucher, R. C. (1995) Science 269, 847-850.
8. Sugita, M., Yue, Y., and Foskett, J. K. (1998) EMBO J. 17, 898-908.
9. Field, M., and Semrad, C. E. (1993) Annu. Rev. Physiol. 55, 631-655.
10. Vaandrager, A. B., Smolenski, A., Tilly, B. C., Houtsmuller, A. B., Ehlert, E. M., Bot, A. G., Edixhoven, M., Boomaars, W. E., Lohmann, S. M., and de Jonge, H. R. (1998) Proc. Natl. Acad. Sci. U.S.A. 95, 1466-1471.
11. Hanrahan, J. W., Mathews, C. J., Grygorczyk, R., Tabcharani, J. A., Grzelczak, Z., Chang, X. B., and Riordan, J. R. (1996) J. Exp. Zool. 275, 283-291.
12. Tabcharani, J. A., Chang, X. B., Riordan, J. R., and Hanrahan, J. W. (1991) Nature 352, 628-631.
13. Chang, X.-B., Tabcharani, J. A., Hou, Y.-X., Jensen, T. J., Kartner, N., Alon, N., Hanrahan, J. W., and Riordan, J. R. (1993) J. Biol. Chem. 268, 11304-11311.
14. Jia, Y., Mathews, C. J., and Hanrahan, J. W. (1997) J. Biol. Chem. 272, 4978-4984.
15. Seibert, F. S., Tabcharani, J. A., Chang, X. B., Dulhanty, A. M., Mathews, C., Hanrahan, J. W., and Riordan, J. R. (1995) J. Biol. Chem. 270, 2158-2162.
16. Mathews, C. J., Tabcharani, J. A., Chang, X. B., Jensen, T. J., Riordan, J. R., and Hanrahan, J. W. (1998) J. Physiol. (London) 508, 365-377.
17. Anderson, M. P., Berger, H. A., Rich, D. P., Gregory, R. J., Smith, A. E., and Welsh, M. J. (1991) Cell 67, 775-784.
18. Gunderson, K. L., and Kopito, R. R. (1995) Cell 82, 231-239.
19. Winter, M. C., Sheppard, D. N., Carson, M. R., and Welsh, M. J. (1994) Biophys. J. 66, 1398-1403.
20. Schultz, B. D., Bridges, R. J., and Frizzell, R. A. (1996) J. Membr. Biol. 151, 63-75.
21. Denning, G. M., Ostedgaard, L. S., and Welsh, M. J. (1992) J. Cell Biol. 118, 551-559.
22. Schultz, B. D., Takahashi, A., Liu, C., Frizzell, R. A., and Howard, M. (1997) Am. J. Physiol. 273, C2080-C2089.
23. Chang, X. B., Hou, Y. X., Jensen, T. J., and Riordan, J. R. (1994) J. Biol. Chem. 269, 18572-18575.
24. Baukrowitz, T., Hwang, T.-C., Nairn, A. C., and Gadsby, D. C. (1994) Neuron 12, 473-482.
25. Cheng, S. H., Rich, D. P., Marshall, J., Gregory, R. J., Welsh, M. J., and Smith, A. E. (1991) Cell 66, 1027-1036.
26. Hwang, T. C., Horie, M., and Gadsby, D. C. (1993) J. Gen. Physiol. 101, 629-650.
27. 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.
28. 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.
29. McDonough, S., Davidson, N., Lester, H. A., and McCarty, N. A. (1994) Neuron 13, 623-634.
30. Sheppard, D. N., Rich, D. P., Ostedgaard, L. S., Gregory, R. J., Smith, A. E., and Welsh, M. J. (1993) Nature 362, 160-164.
31. Sheppard, D. N., Travis, S. M., Ishihara, H., and Welsh, M. J. (1996) J. Biol. Chem. 271, 14995-15001.
32. Ostedgaard, L. S., Rich, D. P., DeBerg, L. G., and Welsh, M. J. (1997) Biochemistry 36, 1287-1294.
33. Wigley, W. C., Vijayakumar, S., Jones, J. D., Slaughter, C., and Thomas, P. J. (1998) Biochemistry 37, 844-853.
34. Akabas, M. H., Stauffer, D. A., Xu, M., and Karlin, A. (1992) Science 258, 307-310.
35. Akabas, M. H., Kaufmann, C., Cook, T. A., and Archdeacon, P. (1994) J. Biol. Chem. 269, 14865-14868.
36. Cheung, M., and Akabas, M. H. (1996) Biophys. J. 70, 2688-2695.
37. Danielson, M. A., Bass, R. B., and Falke, J. J. (1997) J. Biol. Chem. 272, 32878-32888.
38. Cotten, J. F., and Welsh, M. J. (1997) J. Biol. Chem. 272, 25617-25622.
39. Carroll, T. P., Morales, M. M., Fulmer, S. B., Allen, S. S., Flotte, T. R., Cutting, G. R., and Guggino, W. B. (1995) J. Biol. Chem. 270, 11941-11946.
40. Stauffer, D. A., and Karlin, A. (1994) Biochemistry 33, 6840-6849.
41. Takahashi, A., Watkins, S. C., Howard, M., and Frizzell, R. A. (1996) Am. J. Physiol. 271, C1887-C1894.
42. Roberts, D. D., Lewis, S. D., Ballou, D. P., Olson, S. T., and Shafer, J. A. (1986) Biochemistry 25, 5595-5601.
43. Linsdell, P., and Hanrahan, J. W. (1998) J. Gen. Physiol. 111, 601-614.
44. Deisenhofer, J., Epp, O., Miki, K., Huber, R., and Michel, H. (1985) Nature 318, 618-624.
45. Cowan, S. W., Schirmer, T., Rummel, G., Steiert, M., Ghosh, R., Pauptit, R. A., Jansonius, J. N., and Rosenbusch, J. P. (1992) Nature 358, 727-733.
46. Schirmer, T., Keller, T. A., Wang, Y.-F., and Rosenbusch, J. P. (1995) Nature 267, 512-514.
47. Weiss, M. S., Abele, U., Weckesser, J., Welte, W., Schiltz, E., and Schulz, G. E. (1991) Science 254, 1627-1630.
48. Doyle, D. A., Cabral, J. M., Pfuetzner, R. A., Kuo, A., Gulbis, J. M., Cohen, S. L., Chait, B. T., and MacKinnon, R. (1998) Science 280, 69-77.
49. Tsukihara, T., Aoyama, H., Yamashita, E., Tomizaki, T., Yamaguchi, H., Shinzawa-Itoh, K., Nakashima, R., Yaono, R., and Yoshikawa, S. (1996) Science 272, 1136-1144.
50. Koepke, J., Hu, X., Muenke, C., Schulten, K., and Michel, H. (1996) Structure 4, 581-597.
51. Kuhlbrandt, W. (1995) Structure 3, 521-525.
52. Clark, P. L., Liu, Z. P., Zhang, J., and Gierasch, L. M. (1996) Protein Sci. 5, 1108-1117.
53. Fu, D., Ballesteros, J. A., Weinstein, H., Chen, J., and Javitch, J. A. (1996) Biochemistry 35, 11278-11285.
54. Cheung, M., and Akabas, M. H. (1997) J. Gen. Physiol. 109, 289-300.
55. Li, S. C., and Deber, C. M. (1994) Nat. Struct. Biol. 1, 368-373.
56. Li, S. C., Goto, N. K., Williams, K. A., and Deber, C. M. (1996) Proc. Natl. Acad. Sci. U.S.A. 93, 6676-6681.
57. von Heijne, G. (1991) J. Mol. Biol. 218, 499-503.
58. Chillon, M., Casals, T., Nunes, V., Gimenez, J., Perez Ruiz, E., and Estivill, X. (1993) Hum. Mol. Genet. 2, 1741-1742.
59. Ballesteros, J. A., and Weinstein, H. (1992) Biophys. J. 62, 110-111.
60. Audrezet, M. P., Canki-Klain, N., Mercier, B., Bracar, D., Verlingue, C., and Ferec, C. (1994) Hum. Genet. 93, 659-662.
61. Mercier, B., Verlingue, C., Lissens, W., Silber, S. J., Novelli, G., Bonduelle, M., Audrezet, M. P., and Ferec, C. (1995) Am. J. Hum. Genet. 56, 272-277.
62. Claustres, M., Laussel, M., Desgeorges, M., Giansily, M., Culard, J. F., Razakatsara, G., and Demaille, J. (1993) Hum. Mol. Genet. 2, 1209-1213.
63. Morral, N., Dork, T., Llevadot, R., Dziadek, V., Mercier, B., Ferec, C., Costes, B., Girodon, E., Zielenski, J., Tsui, L. C., Tummler, B., and Estivill, X. (1996) Hum. Mutat. 8, 149-159.