Normal gating of CFTR requires ATP binding to both nucleotide-binding domains and hydrolysis at the second nucleotide-binding domain

Proceedings of the National Academy of Sciences of the United States of America

Volumn 102, Issue 2, 2005, Pages 455-460

  Berger, Allan L ^a   Ikuma, Mirtsuhiro ^a   Welsh, Michael J ^a  

^a UNIVERSITY OF IOWA   (United States)

Author keywords

Cystic fibrosis; Ion channel; Walker motif

Indexed keywords

ADENOSINE TRIPHOSPHATE; CHLORIDE CHANNEL; CYSTEINE; LYSINE; N ETHYLMALEIMIDE; PHENYLALANINE; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ARTICLE; CHANNEL GATING; CONTROLLED STUDY; HUMAN; HUMAN CELL; HYDROLYSIS; MUTATION; NUCLEOTIDE BINDING SITE; PRIORITY JOURNAL; SUBSTITUTION REACTION;

ADENOSINE TRIPHOSPHATE; BINDING SITES; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DIMERIZATION; HUMANS; HYDROLYSIS; ION CHANNEL GATING;

EID: 12244313037     PISSN: 00278424     EISSN: None     Source Type: Journal    
DOI: 10.1073/pnas.0408575102     Document Type: Article

References (50)

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., et al. (1989) Science 245, 1066-1073.
2. Welsh, M. J., Ramsey, B. W., Accurso, F. & Cutting, G. R. (2001) in The Metabolic and Molecular Basis of Inherited Disease, eds. Scriver, C. R., Beaudet, A. L., Sly, W. S., Valle, D., Childs, B. & Vogelstein, B. (McGraw-Hill, New York), pp. 5121-5189.
3. Hung, L. W., Wang, I. X., Nikaido, K., Liu, P. Q., Ames, G. F. L. & Kim, S. H. (1998) Nature 396, 703-707.
4. Diederichs, K., Diez, J., Greller, G., Müller, C., Breed, J., Schnell, C., Vonrhein, C., Boos, W. & Welte, W. (2000) EMBO J. 19, 5951-5961.
5. Gaudet, R. & Wiley, D. C. (2001) EMBO J. 20, 4964-4972.
6. Yuan, Y. R., Blecker, S., Martsinkevich, O., Millen, L., Thomas, P. J. & Hunt, J. F. (2001) J. Biol. Chem. 276, 32313-32321.
7. Smith, P. C., Karpowich, N., Millen, L., Moody, J. E., Rosen, J., Thomas, P. J. & Hunt, J. F. (2002) Mol. Cell 10, 139-149.
8. Lewis, H. A., Buchanan, S. G., Burley, S. K., Conners, K., Dickey, M., Dorwart, M., Fowler, R., Gao, X., Guggino, W. B., Hendrickson, W. A., et al. (2004) EMBO J. 23, 282-293.
9. Locher, K. P., Lee, A. T. & Rees, D. C. (2002) Science 296, 1091-1098.
10. Hopfner, K.-P., Karcher, A., Shin, D. S., Craig, L., Arthur, L. M., Carney, J. P. & Tainer, J. A. (2000) Cell 101, 789-800.
11. Randak, C. & Welsh, M. J. (2003) Cell 115, 837-850.
12. Li, C., Ramjeesingh, M., Wang, W., Garami, E., Hewryk, M., Lee, D., Rommens, J. M., Galley, K. & Bear, C. E. (1996) J. Biol. Chem. 271, 28463-28468.
13. Szabo, K., Szakacs, G., Hegeds, T. & Sarkadi, B. (1999) J. Biol. Chem. 274, 12209-12212.
14. Aleksandrov, L., Mengos, A., Chang, X., Aleksandrov, A. & Riordan, J. R. (2001) J. Biol. Chem. 276, 12918-12923.
15. Aleksandrov, L., Aleksandrov, A. A., Chang, X. & Riordan, J. R. (2002) J. Biol. Chem. 277, 15419-15425.
16. Ramjeesingh, M., Li, C., Garami, E., Huan, L. J., Galley, K., Wang, Y. & Bear, C. E. (1999) Biochemistry 38, 1463-1468.
17. Carson, M. R., Travis, S. M. & Welsh, M. J. (1995) J. Biol. Chem. 270, 1711-1717.
18. Gunderson, K. L. & Kopito, R. R. (1995) Cell 82, 231-239.
19. Vergani, P., Nairn, A. C. & Gadsby, D. C. (2003) J. Gen. Physiol. 121, 17-36.
20. Zeltwanger, S., Wang, F., Wang, G. T., Gillis, K. D. & Hwang, T. C. (1999) J. Gen. Physiol. 113, 541-554.
21. Powe, A. C., Jr., Al-Nakkash, L., Li, M. & Hwang, T. C. (2002) J. Physiol. 539, 333-346.
22. Cotten, J. F. & Welsh, M. J. (1998) J. Biol Chem. 273, 31873-31879.
23. Berger, A. L., Ikuma, M., Hunt, J. F., Thomas, P. J. & Welsh, M. J. (2002) J. Biol. Chem. 277, 2125-2131.
24. Cotten, J. F. & Welsh, M. J. (1997) J. Biol. Chem. 272, 25617-25622.
25. Dahan, D., Evagelidis, A., Hanrahan, J. W., Hinkson, D. A., Jia, Y., Luo, J. & Zhu, T. (2001) Pflügers Arch. 443, Suppl. 1, S92-S96.
26. Travis, S. M., Carson, M. R., Ries, D. R. & Welsh, M. J. (1993) J. Biol. Chem. 268, 15336-15339.
27. Ostedgaard, L. S., Rich, D. P., DeBerg, L. G. & Welsh, M. J. (1997) Biochemistry 36, 1287-1294.
28. Cheng, S. H., Gregory, R. J., Marshall, J., Paul, S., Souza, D. W., White, G. A., O'Riordan, C. R. & Smith, A. E. (1990) Cell 63, 827-834.
29. Carter, P., Nilsson, B., Burnier, J. P., Burdick, D. & Wells, J. A. (1989) Proteins 6, 240-248.
30. Al-Shawi, M. K., Urbatsch, I. L. & Senior, A. E. (1994) J. Biol. Chem. 269, 8986-8992.
31. Loo, T. W. & Clarke, D. M. (1995) J. Biol. Chem. 270, 22957-22961.
32. Kidd, J. F., Ramjeesingh, M., Stratford, F., Huan, L. J. & Bear, C. E. (2004) J. Biol. Chem. 279, 41664-41669.
33. Gentzsch, M., Aleksandrov, A., Aleksandrov, L. & Riordan, J. R. (2002) Biochem. J. 366, 541-548.
34. Schneider, E. & Hunke, S. (1998) FEMS Microbiol. Rev. 22, 1-20.
35. Shyamala, V., Baichwal, V., Beall, E. & Ames, G. F. L. (1991) J. Biol. Chem. 266, 18714-18719.
36. Basso, C., Vergani, P., Nairn, A. C. & Gadsby, D. C. (2003) J. Gen. Physiol. 122, 333-348.
37. Panagiotidis, C. H., Reyes, M., Sievertsen, A., Boos, W. & Shuman, H. A. (1993) J. Biol. Chem. 268, 23685-23696.
38. Allard, M., Geoffre, S., Legendre, P., Vincent, J. D. & Simonnet, G. (1989) Brain Res. 500, 169-176.
39. Harrington, M. A., Gunderson, K. L. & Kopito, R. R. (1999) J. Biol. Chem. 274, 27536-27544.
40. Ikuma, M. & Welsh, M. J. (2000) Proc. Natl. Acad. Sci. USA 97, 8675-8680.
41. Aleksandrov, A. A., Chang, X., Aleksandrov, L. & Riordan, J. R. (2000) J. Physiol. 528, 259-265.
42. Venglarik, C. J., Schultz, B. D., Frizzell, R. A. & Bridges, R. J. (1994) J. Gen. Physiol. 104, 123-146.
43. Winter, M. C., Sheppard, D. N., Carson, M. R. & Welsh, M. J. (1994) Biophys. J. 66, 1398-1403.
44. Cai, Z. & Sheppard, D. N. (2002) J. Biol. Chem. 277, 19546-19553.
45. Hennager, D. J., Ikuma, M., Hoshi, T. & Welsh, M. J. (2001) Proc. Natl. Acad. Sci. USA 98, 3594-3599.
46. Dousmanis, A. G., Nairn, A. C. & Gadsby, D. C. (2002) J. Gen. Physiol. 119, 545-559.
47. Mathews, C. J., Tabcharani, J. A. & Hanrahan, J. W. (1998) J. Membr. Biol. 163, 55-66.
48. Aleksandrov, A. A. & Riordan, J. R. (1998) FEBS Lett. 431, 97-101.
49. Ramjeesingh, M., Kidd, J. F., Huan, L. J., Wang, Y. & Bear, C. E. (2003) Biochem. J. 374, 793-797.
50. Raghuram, V., Mak, D. D. & Foskett, J. K. (2001) Proc. Natl. Acad. Sci. USA 98, 1300-1305.