Structure and function of the CFTR chloride channel

Physiological Reviews

Volumn 79, Issue 1 SUPPL. 1, 1999, Pages

  Sheppard, David N ^a   Welsh, Michael J ^a  

^a NONE

Author keywords

[No Author keywords available]

Indexed keywords

ABC TRANSPORTER; ADENOSINE TRIPHOSPHATE; CHLORIDE CHANNEL; CHLORIDE ION; CYCLIC AMP DEPENDENT PROTEIN KINASE; NUCLEOTIDE BINDING PROTEIN; PHOSPHATASE; PROTEIN KINASE (CALCIUM,CALMODULIN) II; PROTEIN TYROSINE KINASE; TRANSMEMBRANE CONDUCTANCE REGULATOR;

CHANNEL GATING; CYSTIC FIBROSIS; DIARRHEA; ELECTROLYTE TRANSPORT; GENE MUTATION; HUMAN; ION CONDUCTANCE; NONHUMAN; PRIORITY JOURNAL; PROTEIN CONFORMATION; PROTEIN DOMAIN; PROTEIN PHOSPHORYLATION; PROTEIN STRUCTURE; REVIEW;

EID: 0032912589     PISSN: 00319333     EISSN: None     Source Type: Journal    
DOI: 10.1152/physrev.1999.79.1.S23     Document Type: Review

References (152)

1. AKABAS, M. H., C. KAUFMANN, T. A. COOK, AND P. ARCHDEACON. Amino acid residues lining the chloride channel of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 269: 14865-14868, 1994.
2. AMES, G. F.-L., C. S. MIMURA, AND V. SHYAMALA. Bacterial periplasmic permeases belong to a family of transport proteins operating from Escherichia coli to human: traffic ATPases. FEMS Microbiol. Rev. 75: 429-446, 1990.
3. ANDERSON, M. P., H. A. BERGER, D. P. RICH, R. J. GREGORY, A. E. SMITH, AND M. J. WELSH. Nucleoside triphosphates are required to open the CFTR chloride channel. Cell 67: 775-784, 1991.
4. ANDERSON, M. P., R. J. GREGORY, S. THOMPSON, D. W. SOUZA, S. PAUL, R. C. MULLIGAN, A. E. SMITH, AND M. J. WELSH. Demonstration that CFTR is a chloride channel by alteration of its anion selectivity. Science 253: 202-205, 1991.
5. ANDERSON, M. P., D. P. RICH, R. J. GREGORY, A. E. SMITH, AND M. J. WELSH. Generation of cAMP-activated chloride currents by expression of CFTR. Science 251: 679-682, 1991.
6. ANDERSON, M. P., AND M. J. WELSH. Calcium and cAMP activate different chloride channels in the apical membrane of normal and cystic fibrosis epithelia. Proc. Natl. Acad. Sci. USA 88: 6003-6007, 1991.
7. ANDERSON, M. P., AND M. J. WELSH. Regulation by ATP and ADP of CFTR chloride channels that contain mutant nucleotide-binding domains. Science 257: 1701-1704, 1992.
8. ANNEREAU, J. P., U. WULBRAND, A. VANKEERBERGHEN, H. CUPPENS, F. BONTEMS, B. TUMMLER, J. J. CASSIMAN, AND V. STOVEN. A novel model for the first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator. FEBS Lett. 407: 303-308, 1997.
9. ARISPE, N., E. ROJAS, J. HARTMAN, E. J. SORSCHER, AND H. B. POLLARD. Intrinsic anion channel activity of the recombinant first nucleotide binding fold domain of the cystic fibrosis transmembrane regulator protein. Proc. Natl. Acad. Sci. USA 89: 1539-1543, 1992.
10. ARREOLA, J., J. E. MELVIN, AND T. BEGENISICH. Volume-activated chloride channels in rat parotid acinar cells. J. Physiol. (Lond.) 184: 677-687, 1995.
11. ASHCROFT, S. J., AND F. M. ASHCROFT. Properties and functions of ATP-sensitive K-channels. Cell Signal. 2: 197-214, 1990.
12. BAUKROWITZ, T., T. C. HWANG, A. C. NAIRN, AND D. C. GADSBY. Coupling of CFTR CI channel gating to an ATP hydrolysis cycle. Neuron 12: 473-482, 1994.
13. BEAR, C. E., F. DUGUAY, A. L. NAISMITH, N. KARTNER, J. W. HANRAHAN, AND J. R. RIORDAN. CI channel activity in Xenopus oocytes expressing the cystic fibrosis gene. J. Biol. Chern. 266: 19142-19145, 1991.
14. BEAR, C. E., C. LI, N. KARTNER, R. J. BRIDGES, T. J. JENSEN, M. RAMJEESINGH, AND J. R. RIORDAN. Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR). Cell 68: 809-818, 1992.
15. BECQ, F., T. J. JENSEN, X.-B. CHANG, A. SAVOIA, J. M. ROMMENS, L.-C. TSUI, M. BUCHWALD, J. R. RIORDAN, AND J. W. HANRAHAN. Phosphatase inhibitors activate normal and defective CFTR chloride channels. Proc. Natl. Acad. Sci. USA 91: 9160-9164, 1994.
16. BERGER, H. A., M. P. ANDERSON, R. J. GREGORY, S. THOMPSON, P. W. HOWARD, R. A. MAURER, R. MULLIGAN, A. E. SMITH, AND M. J. WELSH. Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel. J. Clin. Invest. 88: 1422-1431, 1991.
17. - channel by negative charge in the R domain. Jpn. J. Physiol. 44, Suppl. 2: S167-S172, 1994.
18. - channel by specific protein kinases and protein phosphatases. J. Biol. Chem. 268: 2037-2047, 1993.
19. BIJMAN, J., W. DALEMANS, M. KANSEN, J. KEULEMANS, E. VERBEEK, A. HOOGEVEEN, H. DE-JONGE, M. WILKE, D. DREYER, J. P. LECOCQ, A. PAVIRANI, AND B. SCHOLTE. Low-conductance chloride channels in IEC-6 and CF nasal cells expressing CFTR. Am. J. Physiol. 264 (Lung Cell. Mol. Physiol. 8): L229-L235, 1993.
20. BORMANN, J., O. P. HAMILL, AND B. SAKMANN. Mechanism of anion permeation through channels gated by glycine and gamma-aminobutyric acid in mouse cultured spinal neurones. J. Physiol. (Lond.) 385: 243-286, 1987.
21. + transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation. J. Clin. Invest. 78: 1245-1252, 1986.
22. BRANDL, C. J., AND C. M. DEBER. Hypothesis about the function of membrane-buried proline residues in transport proteins. Proc. Natl. Acad. Sci. USA 83: 917-921, 1986.
23. CARSON, M. R., S. M. TRAVIS, AND M. J. WELSH. The two nucleotide-binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) have distinct functions in controlling channel activity. J. Biol. Chem. 270: 1711-1717, 1995.
24. CARSON, M. R., AND M. J. WELSH. 5′-Adenylylimidodiphosphate does not activate CFTR chloride channels in cell-free patches of membrane. Am. J. Physiol. 265 (Lung Cell. Mol. Physiol. 9): L27-L32, 1993.
25. CARSON, M. R., AND M. J. WELSH. Structural and functional similarities between the nucleotide-binding domains of CFTR and GTP-binding proteins. Biophys. J. 69: 2443-2448, 1995.
26. - channels. J. Biol. Chem. 270: 20466-20472, 1995.
27. CHANG, X.-B., Y.-X. HOU, T. J. JENSEN, AND J. R. RIORDAN. Mapping of cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion. J. Biol. Chem. 269: 18572-18575, 1994.
28. CHANG, X.-B., J. A. TABCHARANI, Y.-X. HOU, T. J. JENSEN, N. KARTNER, N. ALON, J. W. HANRAHAN, AND J. R. RIORDAN. Protein kinase A (PKA) still activates CFTR chloride channel after mutagenesis of all 10 PKA consensus phosphorylation sites. J. Biol. Chem. 268: 11304-11311, 1993.
29. CHEN, M., AND J.-T. ZHANG. Membrane insertion, processing and topology of cystic fibrosis transmembrane conductance regulator (CFTR) in microsomal membranes. Mol. Membr. Biol. 13: 33-40, 1996.
30. CHENG, S. H., D. P. RICH, J. MARSHALL, R. J. GREGORY, M. J. WELSH, AND A. E. SMITH. Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel. Cell 66: 1027-1036, 1991.
31. CHEUNG, M., AND M. H. AKABAS. Identification of cystic fibrosis transmembrane conductance regulator channel-lining residues in and flanking the M6 membrane-spanning segment. Biophys. J. 70: 2688-2695, 1996.
32. CHEUNG, M., AND M. H. AKABAS. Locating the anion-selectivity filter of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel. J. Gen. Physiol. 109: 289-299, 1997.
33. COTTEN, J. F., L. S. OSTEDGAARD, M. R. CARSON, AND M. J. WELSH. Effect of cystic fibrosis-associated mutations in the fourth intracellular loop of cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 271: 21279-21284, 1996.
34. COTTEN, J. F., AND M. J. WELSH. Covalent modification of the regulatory domain irreversibly stimulates cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 272: 25617-25622, 1997.
35. DANI, J. A., J. A. SANCHEZ, AND B. HILLE. Lyotropic anions. Na channel gating and Ca electrode response. J. Gen. Physiol. 81: 255-281, 1983.
36. DELANEY, S. J., D. P. RICH, S. A. THOMSON, M. R. HARGRAVE, P. K. LOVELOCK, M. J. WELSH, AND B. J. WAINWRIGHT. Cystic fibrosis transmembrane conductance regulator splice variants are not conserved and fail to produce chloride channels. Nature Genet. 4: 426-431, 1993.
37. DENNING, G. M., L. S. OSTEDGAARD, S. H. CHENG, A. E. SMITH, AND M. J. WELSH. Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia. J. Clin. Invest. 89: 339-349, 1992.
38. DEVIDAS, S., H. YUE, AND W. B. GUGGINO. The second half of the cystic fibrosis transmembrane conductance regulator forms a functional chloride channel. J. Biol Chem. 273: 29373-29380, 1998
39. DRUMM, M. L., D. J. WILKINSON, L. S. SMIT, R. T. WORRELL, T. V. STRONG, R. A. FRIZZELL, D. C. DAWSON, AND F. S. COLLINS. Chloride conductance expressed by ΔF508 and other mutant CFTRs in Xenopus oocytes. Science 254: 1797-1799, 1991.
40. DULHANTY, A. M., X.-B. CHANG, AND J. R. RIORDAN. Mutation of potential phosphorylation sites in the recombinant R domain of the cystic fibrosis transmembrane conductance regulatory has significant effects on domain conformation. Biochem. Biophys. Res. Commun. 206: 207-214, 1996.
41. DULHANTY, A. M., AND J. R. RIORDAN. Phosphorylation by cAMP-dependent protein kinase causes a conformational change in the R domain of the cystic fibrosis transmembrane conductance regulator. Biochemistry 33: 4072-4079, 1994.
42. DULHANTY, A. M., AND J. R. RIORDAN. A two-domain model for the R domain of the cystic fibrosis transmembrane conductance regulator based on sequence similarities. FEBS Lett. 343: 109-114, 1994.
43. FISCHER, H., AND T. E. MACHEN. CFTR displays voltage dependence and two gating modes during stimulation. J. Gen. Physiol. 104: 541-566, 1994.
44. c-src regulates the fast gate of the cystic fibrosis transmembrane conductance regulator chloride channel. Biophys. J. 71: 3073-3082, 1996.
45. GADSBY, D. C., T.-C. HWANG, M. HORIE, G. NAGEL, AND A. C. NAIRN. Cardiac chloride channels: incremental regulation by phosphorylation/dephosphorylation. Ann. NY Acad. Sci. 707: 259-274, 1993.
46. GADSBY, D. C., G. NAGEL, AND T.-C. HWANG. The CFTR chloride channel of mammalian heart. Annu. Rev. Physiol. 57: 387-416, 1995.
47. GADSBY, D. C., AND A. C. NAIRN. Regulation of CFTR channel gating. Trends Biochem. Sci. 19: 513-518, 1994.
48. GILMAN, A. G. G proteins: transducers of receptor-generated signals. Annu. Rev. Biochem. 56: 615-649, 1987.
49. GOTTESMAN, M. M., S. V. AMBUDKAR, M. M. CORNWELL, I. PASTAN, AND U. A. GERMANN. Multidrug resistance transporter. In: Molecular Biology of Membrane Transport Disorders, edited by S. G. Schultz, T. E. Andreoli, A. M. Brown, D. M. Fambrough, J. F. Hoffman, and M. J. Welsh. New York: Plenum, 1996, p. 243-257.
50. GREGORY, R. J., D. P. RICH, S. H. CHENG, D. W. SOUZA, S. PAUL, P. MANAVALAN, M. P. ANDERSON, M. J. WELSH, AND A. E. SMITH. Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2. Mol. Cell. Biol. 11:3886-3893, 1991.
51. GRYGORCZYK, R., J. A. TABCHARANI, AND J. W. HANRAHAN. CFTR channels expressed in CHO cells do not have detectable ATP conductance. J. Membr. Biol. 151: 139-148, 1996.
52. GUINAMARD, R., AND M. H. AKABAS. Arg352 is a major determinant of charge selectivity in the CFTR chloride channel (Abstract). Biophys. J. 74: A395, 1998.
53. GUNDERSON, K. L., AND R. R. KOPITO. Effects of pyrophosphate and nucleotide analogs suggest a role for ATP hydrolysis in cystic fibrosis transmembrane regulator channel gating. J. Biol. Chem. 269: 19349-19353, 1994.
54. GUNDERSON, K. L., AND R. R. KOPITO. Conformational states of CFTR associated with channel gating: the role ATP binding and hydrolysis. Cell 82: 231-239, 1995.
55. HALM, D. R., AND R. A. FRIZZELL. Anion permeation in an apical membrane chloride channel of a secretory epithelial cell. J. Gen. Physiol. 99: 339-366, 1992.
56. HANRAHAN, J. W., F. DUGUAY, S. SANSOM, N. ALON, T. JENSEN, J. R. RIORDAN, AND Z. GRZELCZAK. Low-conductance chloride channel activated by cAMP in the rectal gland of the shark Squalus acanthias and in cells heterologously expressing shark CFTR. Bull. Mount Desert Island Biol. Lab. 32: 48-49, 1993.
57. HANRAHAN, J. W., J. A. TABCHARANI, AND R. GRYGORCZYK. Patch clamp studies of apical membrane chloride channels. In: Cystic Fibrosis: Current Topics, edited by J. A. Dodge, D. J. H. Brock, and J. H. Widdicombe. New York: Wiley, 1993, p. 93-137.
58. HART, P., J. D. WARTH, P. C. LEVESQUE, M. L. COLLIER, Y. GEARY, B. HOROWITZ, AND J. R. HUME. Cystic fibrosis gene encodes a cAMP-dependent chloride channel in heart. Proc. Natl. Acad. Sci. USA 93: 6343-6348, 1996.
59. HASEGAWA, H., W. SKACH, O. BAKER, M. C. CALAYAG, V. LINGAPPA, AND A. S. VERKMAN. A multifunctional aqueous channel formed by CFTR. Science 258: 1477-1479, 1992.
60. HIGGINS, C. F. ABC transporters: from microorganisms to man. Annu. Rev. Cell Biol. 8: 67-113, 1992.
61. - channels in heart. Am. J. Physiol. 264 (Heart Circ. Physiol. 33): H2214-H2220, 1993.
62. HOSHI, T., W. N. ZAGOTTA, AND R. W. ALDRICH. Biophysical and molecular mechanisms of Shaker potassium channel inactivation. Science 250: 533-538, 1990.
63. - conductance in mammalian heart. J. Gen. Physiol. 101: 629-650, 1993.
64. HWANG, T. C., G. NAGEL, A. C. NAIRN, AND D. C. GADSBY. Regulation of the gating of cystic fibrosis transmembrane conductance regulator Cl channels by phosphorylation and ATP hydrolysis. Proc. Natl. Acad. Sci. USA 91: 4698-4702, 1994.
65. HYDE, S. C., P. EMSLEY, M. J. HARTSHORN, M. M. MIMMACK, U. GILEADI, S. R. PEARCE, M. P. GALLAGHER, D. R. GILL, R. E. HUBBARD, AND C. F. HIGGINS. Structural model of ATP-binding proteins associated with cystic fibrosis, multidrug resistance and bacterial transport. Nature 346: 362-365, 1990.
66. KATP: an inward rectifier subunit plus the sulfonylurea receptor. Science 270: 1166-1170, 1995.
67. ISHIHARA, H., AND M. J. WELSH. Block by MOPS reveals a conformation change in the CFTR pore produced by ATP hydrolysis. Am. J. Physiol. 273 (Cell Physiol. 42): C1278-C1289, 1997.
68. JIA, Y., C. J. MATHEWS, AND J. W. HANRAHAN. Phosphorylation by protein kinase C is required for acute activation of cystic fibrosis transmembrane conductance regulator by protein kinase A. J. Biol. Chem. 272: 4978-4984, 1997.
69. KARTNER, N., J. W. HANRAHAN, T. J. JENSEN, A. L. NAISMITH, S. SUN, C. A. ACKERLEY, E. F. REYES, L.-C. TSUI, J. M. ROMMENS, C. E. BEAR, AND J. R. RIORDAN. Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance. Cell 64: 681-691, 1991.
70. KENNELLY, P. J., AND E. G. KREBS. Consensus sequences as substrate specificity determinants for protein kinases and protein phosphatases. J. Biol. Chem. 266: 15555-15558, 1991.
71. KO, Y. H., AND P. L. PEDERSEN. The first nucleotide binding fold of the cystic fibrosis transmembrane conductance regulator can function as an active ATPase. J. Biol. Chem. 270: 22093-22096, 1995.
72. - channels expressed in mammalian cells. J. Physiol. (Lond.) 508: 379-392, 1998.
73. LI, C., M. RAMJEESINGH, AND C. E. BEAR. Purified cystic fibrosis transmembrane conductance regulator (CFTR) does not function as an ATP channel. J. Biol. Chem. 271: 11623-11626, 1996.
74. LI, C., M. RAMJEESINGH, W. WANG, E. GARAMI, M. HEWRYK, D. LEE, J. M. ROMMENS, K. GALLEY, AND C. E. BEAR. ATPase activity of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 271: 28463-28468, 1996.
75. - channels expressed in a mammalian cell line and its regulation by a critical pore residue. J. Physiol. (Lond.) 496: 687-693, 1996.
76. LINSDELL, P., AND J. W. HANRAHAN. Flickery block of single CFTR chloride channels by intracellular anions and osmolytes. Am. J. Physiol. 271 (Cell Physiol. 40): C628-C634, 1996.
77. LINSDELL, P., J. A. TABCHARANI, AND J. W. HANRAHAN. Multi-ion mechanism for ion permeation and block in the cystic fibrosis transmembrane conductance regulator chloride channel. J. Gen. Physiol. 110: 365-377, 1997.
78. LINSDELL, P., J. A. TABCHARANI, J. M. ROMMENS, Y.-X. HOU, X.-B. CHANG, L.-C. TSUI, J. R. RIORDAN, AND J. W. HANRAHAN. Permeability of wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels to polyatomic anions. J. Gen. Physiol. 110: 355-364, 1997.
79. LINSDELL, P., S.-X. ZHENG, AND J. W. HANRAHAN. Possible contribution of the peptide backbone to CFTR anion selectivity (Abstract). Pediatr. Pulmonol. 14: 214, 1997.
80. LOO, T. W., AND D. M. CLARKE. Functional consequences of proline mutations in the predicted transmembrane domain of P-glycoprotein. J. Biol. Chem. 268: 3143-3149, 1993.
81. MA, J., J. E. TASCH, T. TAO, J. ZHAO, J. XIE, M. L. DRUMM, AND P. B. DAVIS. Phosphorylation-dependent block of cystic fibrosis transmembrane conductance regulator chloride channel by exogenous R domain protein. J. Biol. Chem. 271: 7351-7356, 1996.
82. MA, J., J. ZHAO, M. L. DRUMM, J. XIE, AND P. B. DAVIS. Function of the R domain in the cystic fibrosis transmembrane conductance regulator chloride channel. J. Biol. Chem. 272: 28133-28141, 1997.
83. MANAVALAN, P., D. G. DEARBORN, J. M. MCPHERSON, AND A. E. SMITH. Sequence homologies between nucleotide binding regions of CFTR and G-proteins suggest structural and functional similarities. FEBS Lett. 366: 87-91, 1995.
84. MANSOURA, M. K., S. S. SMITH, A. D. CHOI, N. W. RICHARDS, T. V. STRONG, M. L. DRUMM, F. S. COLLINS, AND D. C. DAWSON. Cystic fibrosis transmembrane conductance regulator (CFTR) anion binding as a probe of the pore. Biophys. J. 74: 1320-1332, 1998.
85. MARSHALL, J., S. FANG, L. S. OSTEDGAARD, C. R. O'RIORDAN, D. FERRARA, J. F. AMARA, H. HOPPE, R. K. SCHEULE, M. J. WELSH, A. E. SMITH, AND S. H. CHENG. Stoichiometry of recombinant cystic fibrosis transmembrane conductance regulator in epithelial cells and its functional reconstitution into cells in vitro. J. Biol. Chem. 269: 2987-2995, 1994.
86. - channel by two closely related arylaminobenzoates. J. Gen. Physiol. 102: 1-23, 1993.
87. MCDONOUGH, S., N. DAVIDSON, H. A. LESTER, AND N. A. MCCARTY. Novel pore-lining residues in CFTR that govern permeation and open-channel block. Neuron 13: 623-634, 1994.
88. MORALES, M. M., T. P. CARROLL, T. MORITA, E. M. SCHWIEBERT, O. DEVUYST, P. D. WILSON, A. G. LOPES, B. A. STANTON, H. C. DIETZ, G. R. CUTTING, AND W. B. GUGGINO. Both the wild type and a functional isoform of CFTR are expressed in kidney. Am. J. Physiol. 270 (Renal Fluid Electrolyte Physiol. 39): F1038-F1048, 1996.
89. OBLATT-MONTAL, M., G. L. REDDY, T. IWAMOTO, J. M. TOMICH, AND M. MONTAL. Identification of an ion channel-forming motif in the primary structure of CFTR, the cystic fibrosis chloride channel. Proc. Natl. Acad. Sci. USA 91: 1495-1499, 1994.
90. OSTEDGAARD, L. S., D. P. RICH, L. G. DEBERG, AND M. J. WELSH. Association of domains within the cystic fibrosis transmembrane conductance regulator. Biochemistry 36: 1287-1294, 1997.
91. OSTEDGAARD, L. S., AND M. J. WELSH. Partial purification of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 267: 26142-26149, 1992.
92. OVERHOLT, J. L., A. SAULINO, M. L. DRUMM, AND R. D. HARVEY. Rectification of whole cell cystic fibrosis transmembrane conductance regulator chloride current. Am. J. Physiol. 268 (Cell Physiol. 37): C636-C646, 1995.
93. PASYK, E. A., AND J. K. FOSKETT. Cystic fibrosis transmembrane conductance regulator-associated ATP and adenosine 3′-phosphate 5′-phosphosulfate channels in endoplasmic reticulum and plasma membranes. J. Biol. Chem. 272: 7746-7751, 1997.
94. PICCIOTTO, M. R., J. A. COHN, G. BERTUZZI, P. GREENGARD, AND A. C. NAIRN. Phosphorylation of the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 267: 12742-12752, 1992.
95. - channels and use of human-Xenopus chimeras to investigate the pore properties of CFTR. J. Biol. Chem. 271: 25184-25191, 1996.
96. QUINTON, P. M., AND M. M. REDDY. Control of CFTR chloride conductance by ATP levels through non-hydrolytic binding. Nature 360: 79-81, 1992.
97. RANDAK, C., P. NETH, E. A. AUERSWALD, C. ECKERSKORN, I. ASSFALG-MACHLEIDT, AND W. MACHLEIDT. A recombinant polypeptide model of the second nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator functions as an active ATPase, GTPase and adenylate kinase. FEBS Lett. 410: 180-186, 1997.
98. - conductance. Am. J. Physiol. 271 (Cell Physiol. 40): C35-C42, 1996.
99. REDDY, M. M., P. M. QUINTON, C. HAWS, J. J. WINE, R. GRYGORCZYK, J. A. TABCHARANI, J. W. HANRAHAN, K. L. GUNDERSON, AND R. R. KOPITO. Failure of the cystic fibrosis transmembrane conductance regulator to conduct ATP. Science 271: 1876-1879, 1996.
100. REISIN, I. L., A. G. PRAT, E. H. ABRAHAM, J. F. AMARA, R. J. GREGORY, D. A. AUSIELLO, AND H. F. CANTIELLO. The cystic fibrosis transmembrane conductance regulator is a dual ATP and chloride channel. J. Biol. Chem. 269: 20584-20591, 1994.
101. - channel by negative charge in the R domain. J. Biol. Chem. 268: 20259-20267, 1993.
102. RICH, D. P., R. J. GREGORY, M. P. ANDERSON, P. MANAVALAN, A. E. SMITH, AND M. J. WELSH. Effect of deleting the R domain on CFTR-generated chloride channels. Science 253: 205-207, 1991.
103. RICH, D. P., R. J. GREGORY, S. H. CHENG, A. E. SMITH, AND M. J. WELSH. Effect of deletion mutations on the function of CFTR chloride channels. Receptors Channels 1: 221-232, 1993.
104. RICHARDSON, J. S., AND D. C. RICHARDSON. Principles and patterns of protein conformation. In: Prediction of Protein Structure and the Principles of Protein Conformation, edited by G. D. Fasman. New York: Plenum, 1989, p. 1-98.
105. RIORDAN, J. R., J. M. ROMMENS, B.-S. KEREM, N. ALON, R. ROZMAHEL, Z. GRZELCZAK, J. ZIELENSKI, S. LOK, N. PLAVSIC, J.-L. CHOU, M. L. DRUMM, M. C. IANNUZZI, F. S. COLLINS, AND L.-C. TSUI. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245: 1066-1073, 1989.
106. ROSENBERG, M. F., R. CALLAGHAN, R. C. FORD, AND C. F. HIGGINS. Structure of the multidrug resistance P-glycoprotein to 2.5 nm resolution determined by electron microscopy and image analysis. J. Biol. Chem. 272: 10685-10694, 1997.
107. SCHULTZ, B. D., R. J. BRIDGES, AND R. A. FRIZZELL. Lack of conventional ATPase properties in CFTR chloride channel gating. J. Membr. Biol. 151: 63-75, 1996.
108. SCHULTZ, B. D., A. D. G. DEROOS, C. J. VENGLARIK, A. K. SINGH, R. A. FRIZELL, AND R. J. BRIDGES. Glibenclamide blockade of CFTR chloride channels. Am. J. Physiol. 271 (Lung Cell. Mol. Physiol. 15): L192-L200, 1996.
109. SCHULTZ, B. D., A. TAKAHASHI, C. LIU, R. A. FRIZZELL, AND M. HOWARD. Flag epitope positioned in an external loop preserves normal biophysical properties of CFTR. Am. J. Physiol. 273 (Cell Physiol. 42): C2080-C2089, 1997.
110. - channel gating by ADP and ATP analogues. J. Gen. Physiol. 105: 329-361, 1995.
111. SCHWIEBERT, E. M., M. E. EGAN, T.-H. HWANG, S. B. FULMER, S. S. ALLEN, G. R. CUTTING, AND W. B. GUGGINO. CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP. Cell 81: 1063-1073, 1995.
112. SEIBERT, F. S., Y. JIA, C. J. MATHEWS, J. W. HANRAHAN, J. R. RIORDAN, R. W. LOO, AND D. M. CLARKE. Disease-associated mutations in cytoplasmic loops 1 and 2 of cystic fibrosis transmembrane conductance regulator impede processing or opening of the channel. Biochemistry 36: 11966-11974, 1997.
113. SEIBERT, F. S., P. LINSDELL, T. W. LOO, J. W. HANRAHAN, D. M. CLARKE, AND J. R. RIORDAN. Disease-associated mutations in the fourth cytoplasmic loop of cystic fibrosis transmembrane conductance regulator compromise biosynthetic processing and chloride channel activity. J. Biol. Chem. 271: 15139-15145, 1996.
114. SEIBERT, F. S., P. LINSDELL, T. W. LOO, J. W. HANRAHAN, J. R. RIORDAN, AND D. M. CLARKE. Cytoplasmic loop three of cystic fibrosis transmembrane conductance regulator contributes to regulation of chloride channel activity. J. Biol. Chem. 271: 27493-27499, 1996.
115. SEIBERT, F. S., J. A. TABCHARANI, X. B. CHANG, A. M. DULHANTY, C. MATHEWS, J. W. HANRAHAN, AND J. R. RIORDAN. cAMP-dependent protein kinase-mediated phosphorylation of cystic fibrosis transmembrane conductance regulator residue Ser-753 and its role in channel activation. J. Biol. Chem. 270: 2158-2162, 1995.
116. - channel. Cell 76: 1091-1098, 1994.
117. - channels with altered pore properties. Nature 362: 160-164, 1993.
118. - channels expressed in a murine cell line. J. Physiol. (Lond.) 503: 333-346, 1997.
119. SHEPPARD, D. N., S. M. TRAVIS, H. ISHIHARA, AND M. J. WELSH. Contribution of proline residues in the membrane-spanning domains of CFTR to chloride channel function. J. Biol. Chem. 271: 14995-15001, 1996.
120. + channel regulators on CFTR chloride currents. J. Gen. Physiol. 100: 573-591, 1992.
121. SHUMAN, H. A., AND C. H. PANAGIOTIDIS. Tinkering with transporters: periplasmic binding protein-dependent maltose transport in E. coli. J. Bioenerg. Biomembr. 25: 613-620, 1993.
122. SMIT, L. S., D. J. WILKINSON, M. K. MANSOURA, F. S. COLLINS, AND D. C. DAWSON. Functional roles of the nucleotide-binding folds in the activation of the cystic fibrosis transmembrane conductance regulator. Proc. Natl. Acad. Sci. USA 90: 9963-9967, 1993.
123. SMITH, S. S., E. D. STEINLE, V. PECORARO, M. E. MEYERHOFF, AND D. C. DAWSON. CFTR: probing the pore with permeant ions (Abstract). Pediatr. Pulmonol. 14: 220, 1997.
124. STURGESS, N. C., M. L. ASHFORD, D. L. COOK, AND C. N. HALES. The sulphonylurea receptor may be an ATP-sensitive potassium channel. Lancet 2: 474-475, 1985.
125. - channel in CHO cells stably expressing the cystic fibrosis gene. Nature 352: 628-631, 1991.
126. TABCHARANI, J. A., P. LINSDELL, AND J. W. HANRAHAN. Halide permeation in wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels. J. Gen. Physiol. 110: 341-354, 1997.
127. TABCHARANI, J. A., W. LOW, D. ELIE, AND J. W. HANRAHAN. Low-conductance chloride channel activated by cAMP in the epithelial cell line T84. FEBS Lett. 270: 157-164, 1990.
128. TABCHARANI, J. A., J. M. ROMMENS, Y.-X. HOU, X.-B. CHANG, L.-C. TSUI, J. R. RIORDAN, AND J. W. HANRAHAN. Multi-ion pore behaviour in the CFTR chloride channel. Nature 366: 79-82, 1993.
129. --secreting epithelia and 3T3 fibroblasts. J. Biol. Chem. 269: 17853-17857, 1994.
130. TAO, T., J. XIE, M. L. DRUMM, J. ZHAO, P. B. DAVIS, AND J. MA. Slow conversions among subconductance states of cystic fibrosis transmembrane conductance regulator chloride channel. Biophys. J. 70: 743-753, 1996.
131. TEEM, J. L., H. A. BERGER, L. S. OSTEDGAARD, D. P. RICH, L.-C. TSUI, AND M. J. WELSH. Identification of revertants for the cystic fibrosis ΔF508 mutation using STE6-CFTR chimeras in yeast. Cell 73: 335-346, 1993.
132. TEEM, J. L., M. R. CARSON, AND M. J. WELSH. Mutation of R555 in CFTR-ΔF508 enhances function and partially corrects defective processing. Receptors Channels 4: 63-72, 1996.
133. TILLY, B. C., M. C. WINTER, L. S. OSTEDGAARD, C. O'RIORDAN, A. E. SMITH, AND M. J. WELSH. Cyclic AMP-dependent protein kinase activation of cystic fibrosis transmembrane conductance regulator chloride channels in planar lipid bilayers. J. Biol. Chem. 267: 9470-9473, 1992.
134. TIZIANA-CARROLL, P., M. M. MORALES, S. B. FULMER, S. S. ALLEN, T. R. FLOTTE, G. R. CUTTING, AND W. B. GUGGINO. Alternate translation initiation codons can create functional forms of cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 270: 11941-11946, 1995.
135. TOWNSEND, R. R., P. H. LIPNIUNAS, B. M. TULK, AND A. S. VERKMAN. Identification of protein kinase A phosphorylation sites on NBD1 and R domains of CFTR using electrospray mass spectrometry with selective phosphate ion monitoring. Protein Sci. 5: 1865-1873, 1996.
136. TRAVIS, S. M., H. A. BERGER, AND M. J. WELSH. Protein phosphatase 2C dephosphorylates and inactivates cystic fibrosis transmembrane conductance regulator. Proc. Natl. Acad. Sci. USA 94: 11055-11060, 1997.
137. TUCKER, S. J., D. TANNAHILL, AND C. F. HIGGINS. Identification and developmental expression of the Xenopus laevis cystic fibrosis transmembrane conductance regulator gene. Hum. Mol. Genet. 1: 77-82, 1992.
138. - channel activation (Abstract). Pediatr. Pulmonol. 14: 213, 1997.
139. - channels. Biophys. J. 70: 2696-2703, 1996.
140. VENGLARIK, C. J., B. D. SCHULTZ, R. A. FRIZZELL, AND R. J. BRIDGES. ATP alters current fluctuations of cystic fibrosis transmembrane conductance regulator: evidence for a three-state activation mechanism. J. Gen. Physiol. 104: 123-146, 1994.
141. WELSH, M. J., AND A. E. SMITH. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell 73: 1251-1254, 1993.
142. WELSH, M. J., L.-C. TSUI, T. F. BOAT, AND A. L. BEAUDET. Cystic fibrosis. In: The Metabolic and Molecular Basis of Inherited Disease, edited by C. R. Scriver, A. L. Beaudet, W. S. Sly, and D. Valle. New York: McGraw-Hill, 1995, p. 3799-3876.
143. WILKINSON, D. J., M. K. MANSOURA, P. Y. WATSON, L. S. SMIT, F. S. COLLINS, AND D. C. DAWSON. CFTR: the nucleotide binding folds regulate the accessibility and stability of the activated state. J. Gen. Physiol. 107: 103-119, 1996.
144. WILKINSON, D. J., T. V. STRONG, M. K. MANSOURA, D. L. WOOD, S. S. SMITH, F. S. COLLINS, AND D. C. DAWSON. CFTR activation: additive effects of stimulatory and inhibitory phosphorylation sites in the R domain. Am. J. Physiol. 273 (Lung Cell. Mol. Physiol. 17): L127-L133, 1997.
145. - channels: a kinetic analysis of channel regulation. Biophys. J. 66: 1398-1403, 1994.
146. WINTER, M. C., AND M. J. WELSH. Stimulation of CFTR activity by its phosphorylated R domain. Nature 389: 294-296, 1997.
147. WRIGHT, E. M., AND J. M. DIAMOND. Anion selectivity in biological systems. Physiol. Rev. 57: 109-156, 1977.
148. XIE, J., M. L. DRUMM, J. MA, AND P. B. DAVIS. Intracellular loop between transmembrane segments IV and V of cystic fibrosis transmembrane conductance regulator is involved in regulation of chloride channel conductance state. J. Biol. Chem. 270: 28084-28091, 1995.
149. XIE, J., M. L. DRUMM, J. ZHAO, J. MA, AND P. B. DAVIS. Human epithelial cystic fibrosis transmembrane conductance regulator without exon 5 maintains partial chloride channel function in intracellular membranes. Biophys. J. 71: 3148-3156, 1996.
150. XU, M., AND M. H. AKABAS. Amino acids lining the channel of the γ-aminobutyric acid type A receptor identified by cysteine substitution. J. Biol. Chem. 268: 21505-21508, 1993.
151. ZAGOTTA, W. N., T. HOSHI, AND R. W. ALDRICH. Restoration of inactivation in mutants of Shaker potassium channels by a peptide derived from ShB. Science 250: 568-571, 1990.
152. ZHAO, J., J. XIE, P. B. DAVIS, AND J. MA. Activation of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel by cyclophilin A (Abstract). Pediatr. Pulmonol. 14: 241, 1997.