CFTR is a conductance regulator as well as a chloride channel

Physiological Reviews

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

  Schwiebert, Erik M ^a   Benos, Dale J ^a   Egan, Marie E ^a   Stutts, M Jackson ^a   Guggino, William B ^a  

^a NONE

Author keywords

[No Author keywords available]

Indexed keywords

ABC TRANSPORTER; ACTIN; ADENOSINE TRIPHOSPHATE; CHLORIDE CHANNEL; CHLORIDE ION; CYCLIC AMP DEPENDENT PROTEIN KINASE; GLIBENCLAMIDE; INSULIN; PHOSPHOLIPASE; PHOSPHOLIPID; POTASSIUM CHANNEL; POTASSIUM ION; PROTEIN KINASE C; PURINE RECEPTOR; SODIUM CHANNEL; SODIUM ION; SULFONYLUREA RECEPTOR; TRANSMEMBRANE CONDUCTANCE REGULATOR;

CYSTIC FIBROSIS; DISEASE SEVERITY; HUMAN; KIDNEY MEDULLA; LIPID BILAYER; LUNG DISEASE; MAMMAL CELL; NONHUMAN; OOCYTE; PANCREAS ISLET BETA CELL; PRIORITY JOURNAL; PROTEIN PHOSPHORYLATION; PROTEIN PROTEIN INTERACTION; RESPIRATORY EPITHELIUM; REVIEW;

EID: 0032956071     PISSN: 00319333     EISSN: None     Source Type: Journal    
DOI: 10.1152/physrev.1999.79.1.S145     Document Type: Review

References (140)

1. 2Y purinoceptors. Pharmacol. Ther. 64: 445-475, 1994.
2. ABRAHAM, E. H., P. OKUNIEFF, S. SCALA, P. VOS, M. J. S. OOSTERVELD, A. Y. CHEN, AND B. SHRIVASTAV. Cystic fibrosis trans-membrane conductance regulator and adenosine triphosphate. Science 275: 1324-1325, 1997.
3. ABRAHAM, E. H., A. G. PRAT, L. GERWECK, T. SENEVERATNE, R. J. ARCECI, R. KRAMER, G. GUIDOTTI, AND H. F. CANTIELLO. The multidrug resistance (mdr1) gene product functions as an ATP channel. Proc. Natl. Acad. Sci. USA 90: 312-316, 1993.
4. AGUILAR-BRYAN, L., C. G. NICHOLS, S. W. WECHSLER, J. P. CLEMENT, A. E. BOYD, G. GONZALEZ, H. HERRERA, K. NGUY, J. BRYAN, AND D. A. NELSON. Cloning of the β cell high affinity sulfonylurea receptor: a regulator of insulin secretion. Science 268: 423-426, 1995.
5. AL-BAZZAZ, F. J., AND G. CHENG. Effect of catecholamines on ion transport in dog tracheal epithelium. Appl. Physiol. 47: 397-401, 1979.
6. AMMALA, C., A. MOORHOUSE, F. GRIBBLE, R. ASHFIELD, P. PROKS, P. A. SMITH, H. SAKURA, B. COLES, S. J. H. ASHCROFT, AND F. M. ASHCROFT. Promiscuous coupling between the sulfonylurea receptor and inwardly rectifying potassium channels. Nature 379: 545-548, 1996.
7. 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.
8. + channel complex contains the pertussis toxin-sensitive Galphai-3 protein. J. Biol. Chem. 267: 4759-4765, 1992.
9. BARASCH, J., AND Q. AL-AWQATI. Defective acidification of the biosynthetic pathway in cystic fibrosis. J. Cell Sci. 17: 229-233, 1993.
10. BARASCH, J., B. KISS, A. PRINCE, L. SAIMAN, D. C. GRUENERT, AND Q. AL-AWQATI. Defective acidification of intracellular organelles in cystic fibrosis. Nature 352: 70-73, 1991.
11. BARNARD, E. A., G. BURNSTOCK, AND T. E. WEBB. G protein-coupled receptors for ATP and other nucleotides: a new receptor family. Trends Pharmacol. Sci. 15: 67-70, 1994.
12. 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.
13. BEAUDET, L., AND P. GROS. Functional dissection of P-glycoprotein nucleotide-binding domains in chimeric and mutant proteins. J. Biol. Chem. 270: 17159-17170, 1995.
14. BENNETT, W. D., K. N. OLIVIER, K. L. ZEMAN, K. W. HOHNEKER, R. C. BOUCHER, AND M. R. KNOWLES. Effect of uridine 5'-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis. Am. J. Respir. Crit. Care Med. 153: 1796-1801, 1996.
15. BERDIEV, B. K., A. G. PRAT, H. F. CANTIELLO, D. A. AUSIELLO, C. M. FULLER, B. JOVOV, D. J. BENOS, AND I.I. ISMAILOV. Regulation of epithelial sodium channels by short actin filaments. J. Biol. Chem. 271: 17704-17710, 1996.
16. BIRNBAUMER, L., J. ABRAMOWITZ, A. YATANI, K. OKABE, R. MATTERA, R. GRAF, J. SANFORD, J. CODINA, AND A. M. BROWN. Roles of G proteins in coupling receptors to ionic channels and other effector systems. CRC Crit. Rev. Biochem. Mol. Biol. 25: 225-244, 1990.
17. BIRNBAUMER, L., AND A. M. BROWN. Ionic channels and their regulation by G protein subunits. Annu. Rev. Physiol. 52: 197-213, 1990.
18. + channel. II. Cloning and distribution of alternative forms. Am. J. Physiol. 268 (Renal Fluid Electrolyte Physiol. 37): F1132-F1140, 1995.
19. BOUCHER, R. C. Chemical modulation of airway epithelial permeability. Environ. Health Perspect. 35: 3-12, 1980.
20. BOUCHER, R. C. Human airway ion transport. Am. J. Respir. Crit. Care Med. 150: 271-281, 1994.
21. BOUCHER, R. C., AND J. T. GATZY. Regional effects of autonomic agents on ion transport across excised canine airways. J. Appl. Physiol. 52: 893-901, 1982.
22. BOUCHER, R. C., M. J. STUTTS, AND J. T. GATZY. Regional differences in bioelectric properties and ion flow in excised canine airways. J. Appl. Physiol. 51: 706-714, 1981.
23. BOUCHER, R. C., M. J. STUTTS, M. R. KNOWLES, L. CANTLEY, AND J. T. GATZY. Sodium transport in cystic fibrosis epithelia. J. Clin. Invest. 78: 1245-1252, 1986.
24. + transport in cystic fibrosis epithelia. Abnormal basal rate and response to adenylate cyclase activation. J. Clin. Invest. 78: 1245-1252, 1986.
25. BRADBURY, N. A., T. JILLING, G. BERTA, E. J. SORSCHER, R. J. BRIDGES, AND K. L. KIRK. Regulation of plasma membrane recycling by CFTR. Science 256: 530-532, 1992.
26. 2+ channels. Ann. NY Acad. Sci. 237: F121-F127, 1989.
27. + channel subunits in normal and cystic fibrosis airways. Am. J. Physiol, 269 (Cell Physiol. 38): C511-C518, 1995.
28. CANESSA, C. M., J.-D. HORISBERGER, AND B. C. ROSSIER. Epithelial sodium channel related to proteins involved in neurodegradation. Nature 361: 467-470, 1993.
29. + channel is made of three homologous subunits. Nature 367: 463-467, 1994.
30. CANTIELLO, H. F. Role of the actin cytoskeleton in the regulation of the cystic fibrosis transmembrane conductance regulator. Exp. Physiol. 81: 505-514, 1996.
31. 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.
32. 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.
33. CHINET, T. C., J. M. FULLTON, J. R. YANKASKAS, R. C. BOUCHER, AND M. J. STUTTS. Mechanism of sodium hyperabsorption in cultured cystic fibrosis nasal epithelium: a patch-clamp study. Am. J. Physiol. 266 (Cell Physiol. 35): C1061-C1068, 1994.
34. CLANCY, J. P., J. HONG, Z. BEBOK, S. KING, S. DEMOLOMBE, D. ESCANDE, M. HOWARD, AND E. J. SORSCHER. Functional analysis of the CFTR truncation mutations R553X and G542X and wildtype CFTR nucleotide-binding domain-1 (NBD-1) in mammalian cell lines (Abstract). Pediatr. Pulmonol. 12, Suppl: 1, 1995.
35. K opens atrial potassium channels. Science 236: 442-445, 1987.
36. CRAWFORD, I., P. C. MALONEY, P. L. ZEITLIN, W. B. GUGGINO, S. C. HYDE, H. TURLEY, K. C. GATTER, A. HARRIS, AND C. F. HIGGINS. Immunocytochemical localization of the cystic fibrosis gene product CFTR. Proc. Natl. Acad. Sci. USA 88: 9262-9266, 1991.
37. - channel from bovine trachea. J. Biol Chem. 270: 31016-31026, 1995.
38. DEVUYST, O., C. R. BURROW, E. M. SCHWIEBERT, W. B. GUGGINO, AND P. D. WILSON. Developmental regulation of CFTR expression during human nephrogenesis. Am. J. Physiol. 271 (Renal Fluid Electrolyte Physiol. 40): F723-F735, 1996.
39. DRUMM, M. L., H. A. POPE, W. H. CLIFF, J. M. ROMMENS, S. A. MARVIN, L.-C. TSUI, F. S. COLLINS, R. A. FRIZZELL, AND J. M. WILSON. Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer. Cell 62: 1227-1233, 1990.
40. - channels by protein kinase A corrected by insertion of CFTR. Nature 358: 581-584, 1992.
41. + channels. Cell Physiol. Biochem. 6: 195-209, 1996.
42. FREDHOLM, B. B., M. P. ABBRACCHIO, G. BURNSTOCK, J. W. DALY, T. K. HARDEN, K. A. JACOBSON, P. LEFF, AND M. WILLIAMS. Nomenclature and classification of purinoceptors. Pharmacol. Rev. 46: 143-156, 1994.
43. FULMER, S. B., E. M. SCHWIEBERT, M. M. MORALES, W. B. GUGGINO, AND G. R. CUTTING. Two cystic fibrosis transmembrane conductance regulator mutations have different effects on both pulmonary phenotype and regulation of outwardly rectified chloride currents. Proc. Natl. Acad. Sci. USA 92: 6832-6836, 1995.
44. GABRIEL, S. E., L. L. CLARKE, R. C. BOUCHER, AND M. J. STUTTS. CFTR and outwardly rectifying chloride channels are distinct proteins with a regulatory relationship. Nature 363: 263-268, 1993.
45. GALIETTA, L. J., A. RASOLA, M. RUGOLO, M. ZOTTINI, T. MASTROCOLA, D. C. GRUENERT, AND G. ROMEO. Extracellular 2-chloroadenosine and ATP stimulate volume-sensitive chloride current and calcium mobilization in human tracheal 9HTEo cells. FEBS Lett. 304: 61-65, 1992.
46. GALIETTA, L. J., O. ZEGARRA-MORAN, T. MASTROCOLA, C. WOHRLE, M. RUGOLO, AND G. ROMEO. Activation of calcium-dependent potassium and chloride currents by UTP and ATP in CFPAC-1 cells. Pflügers Arch. 426: 534-541, 1994.
47. GILL, D. R., S. C. HYDE, C. F. HIGGINS, M. A. VALVERDE, G. M. MINTENIG, AND F. V. SEPULVEDA. Separation of drug transport and chloride channel functions of the human multidrug resistance P-glycoprotein. Cell 71: 23-32, 1992.
48. GORDON, J. L. Extracellular ATP: effects, sources and fate. Biochem. J. 233: 309-319, 1986.
49. + conductance of rat co-Ionic crypt cells is suppressed by forskolin. Pflügers Arch. 431; 984-986, 1996.
50. GROS, P., AND S. RUETZ. Phosphatidylcholine translocase: a physiological role for the mdr2 gene. Cell 77: 1071-1081, 1994.
51. GRYGORCZYK, R., AND J. W. HANRAHAN. CFTR-independent ATP release from epithelial cells triggered by mechanical stimuli. Am. J. Physiol. 272 (Cell Physiol. 41): C1058-C1066, 1997.
52. GRYGORCZYK, R., AND J. W. HANRAHAN. Cystic fibrosis transmembrane conductance regulator and adenosine triphosphate. Science 275: 1325-1326, 1997.
53. 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.
54. 2+ channel of skeletal muscle. J. Biol. Chem. 266: 19528-19535, 1991.
55. HIGGINS, C. F. ABC transporters: from microorganisms to man. Annu. Rev. Cell Biol. 8: 67-113, 1992.
56. HIGGINS, C. F. The ABC of channel regulation. Cell 82: 693-696, 1995.
57. + channel. Nature 362: 31-38, 1993.
58. HUMMLER, E., P. BARKER, J. T. GATZY, F. BEERMANN, C. VERDUMO, A. SCHMIDT, R. C. BOUCHER, AND B. C. ROSSIER. Early death due to defective neonatal lung liquid clearance in alphaENaC-deficient mice. Nature Genet. 12: 325-328, 1996.
59. HWANG, T.-C., L. LU, P. L. ZEITLIN, D. C. GRUENERT, R. HUGANIR, AND W. B. GUGGINO. Chloride channels in CF: lack of activation by protein kinase C and cAMP-dependent protein kinase. Science 244: 1351-1353, 1989.
60. HWANG, T-H., E. M. SCHWIEBERT, .AND W. B. GUGGINO. Apical and basolateral ATP stimulate tracheal epithelial chloride secretion via multiple purinergic receptors and signaling pathways. Am. J. Physiol, 270 (Cell Physiol. 39): C1611-C1623, 1996.
61. IMUNDO, L., J. BARASCH, A. PRINCE, AND Q. AL-AWQATI. Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. Proc. Natl. Acad. Sci. USA 92: 3019-3025, 1995.
62. K(ATP): an inward rectifier subunit plus the sulfonylurea receptor. Science 270: 1166-1170, 1995.
63. + channels. Neuron 16: 1011-1017, 1996.
64. ISMAILOV, I. I., M. S. AWAYDA, B. JOVOV, B. K. BERDIEV, C. M. FULLER, J. R. DEDMAN, M. KAETZEL, AND D. J. BENOS. Regulation of epithelial sodium channels by the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 271: 4725-4732, 1996.
65. + channels in Liddle's disease. Am. J. Physiol. 270 (Cell Physiol. 39): C214-C223, 1996.
66. + channel. J. Biol. Chem. 271: 24321-24324, 1996.
67. JETTEN, A. M., J. R. YANKASKAS, M. J. STUTTS, N. J. WILLUMSEN, AND R. C. BOUCHER. Persistence of abnormal chloride conductance regulation in transformed cystic fibrosis epithelia. Science 244: 1472-1475, 1989.
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. JOHNSON, L. G., S. E. BOYLES, J. M. WILSON, AND R. C. BOUCHER. Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelia cells. J. Clin. Invest. 95: 1377-1382, 1995.
70. JONES, K. M., E. LIAO, K. W. HOHNEKER, S. TURPIN, M. M. HENNY, K. SELINGER, P. H. HSYU, R. C. BOUCHER, M. R. KNOWLES, AND G. E. DUKES. Pharmacokinetics of amiloride after inhalation and oral administration in adolescents and adults with cystic fibrosis. Pharmacotherapy 17: 263-270, 1997.
71. JORISSEN, M., J. VEREECKE, E. CARMELIET, H. VAN DER BERGHE, AND J.-J. CASSIMAN. Identification of a voltage-and calcium-dependent non-selective cation channel in cultured adult and fetal human nasal epithelial cells. Pflügers Arch. 415: 617-623, 1990.
72. JOVOV, B., I. I. ISMAILOV, AND D. J. BENOS. Cystic fibrosis transmembrane conductance regulator is required for protein kinase A activation of an outwardly rectified anion channel purified from bovine tracheal epithelia. J. Biol. Chem. 270: 1521-1528, 1995.
73. KAWASAKI, M., M. SUZUKI, S. UCHIDA, S. SASAKI, AND F. MARUMO. Stable and functional expression of the ClC-3 chloride channel in somatic cell lines. Neuron 14: 1285-1291, 1995.
74. KEREM, B.-S., J. M. ROMMENS, J. A. BUCHANAN, D. MARKIEWICZ, T. K. COX, A. CHAKRAVARTI, M. BUCHWALD, AND L.-C. TSUI. Identification of the cystic fibrosis gene: genetic analysis. Science 245: 1073-1080, 1989.
75. KNOWLES, M. R. Pharmacologic treatment of abnormal ion transport in the airway epithelium in cystic fibrosis. Chest 107: 71S-76S, 1995.
76. KNOWLES, M. R., M. J. STUTTS, A. SPOCK, N. FISCHER, J. T. GATZY, AND R. C. BOUCHER. Abnormal ion permeation through cystic fibrosis respiratory epithelium. Science 221: 1067-1070, 1983.
77. + currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator. FEBS Lett. 400: 341-344, 1997.
78. LEWIS, D. L, J. D. LECHLEITER, D. KIM, C. NANAVATI, AND D. E. CLAPHAM. Intracellular regulation of ion channels in cell membranes. Mayo Clin. Proc. 65: 1127-1143, 1990.
79. LI, C., M. RAMJESSINGH, 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.
80. LI, M., J. D. McCANN, C. M. LIEDTKE, A. C. NAIRN, P. GREENGARD, AND M. J. WELSH. Cyclic AMP-dependent protein kinase opens chloride channels in normal but not cystic fibrosis airway epithelium. Nature 331: 358-360, 1988.
81. LINSDELL, P., AND J. W. HANRAHAN. Adenosine triphosphate-dependent asymmetry of anion permeation in the cystic fibrosis transmembrane conductance regulator chloride channel. J. Gen. Physiol. 111: 601-604, 1998.
82. LING, B., J. B. ZUCKERMAN, C. LIN, B. J. HARTE, K. A. McNULTY, P. R. SMITH, L. M. GOMEZ, R. T. WORRELL, D. C. EATON, AND T. R. KLEYMAN. Expression of the cystic fibrosis phenotype in a renal amphibian epithelial cell line. J. Biol. Chem. 272: 594-600, 1997.
83. + currents. Am. J. Physiol. 271 (Cell Physiol. 40): C1565-C1573, 1996.
84. + channels in A6 distal nephron cells. Am. J. Physiol, 270 (Renal Fluid Electrolyte Physiol. 39): F798-F805, 1996.
85. MANSOURA, M. K., T. V. STRONG, F. S. COLLINS, AND D. C. DAWSON. A disease-related mutation in the fifth putative transmembrane segment alters the conduction and gating properties of CFTR (Abstract). J. Gen. Physiol. 104: 36a, 1994.
86. + channels. Am. J. Physiol. 260 (Cell Physiol. 29): C1071-C1084, 1991.
87. + channels. Am. J. Physiol. 264 (Lung Cell Mol. Physiol. 8): L357-L364, 1993.
88. + channels (ROMK2) to the inhibitory sulfonylurea compound, glibenclamide, is enhanced by co-expression with the ATP-binding cassette transporter CFTR. Proc. Natl Acad. Sci. USA 93: 8083-8088, 1996.
89. McNICHOLAS, C. M., M. W. NASON, W. B. GUGGINO, E. M. SCHWIEBERT, S. C. HEBERT, G. H. GIEBISCH, AND M. E. EGAN. Sulfonylurea sensitivity of ROMK2 is altered by coexpression with full length and truncated forms of CFTR (Abstract). J. Am. Soc. Nephrol. 7: 1286, 1996.
90. McNICHOLAS, C. M., M. W. NASON, W. B. GUGGINO, E. M. SCHWIEBERT, S. C. HEBERT, G. H. GIEBISCH, AND M. E. EGAN. A functional CFTR-NBF1 is required for ROMK2-CFTR interaction. Am. J. Physiol. 273 (Renal Physiol 42): F843-F848, 1997.
91. 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 wild-type and a functional isoform of CFTR are expressed in kidney. Am. J. Physiol. 270 (Renal Fluid Electrolyte Physiol. 39): F1038-F1048, 1996.
92. PALMER, L. G. Epithelial Na+ channels: function and diversity. Annu. Rev. Physiol. 54: 51-66, 1992.
93. 2+ release and influx in polarized epithelia. Nature 377: 643-646, 1995.
94. 2V nucleotide receptor, a target for cystic fibrosis pharmacotherapy. Proc. Natl. Acad. Sci. USA 91: 3275-3279, 1994.
95. 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. 273: 7746-7751, 1997.
96. + channels. Science 268: 372-373, 1995.
97. PIAZZA-CARROLL, T., 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 the cystic fibrosis transmembrane conductance regulator. J. Biol. Chem. 270: 11941-11946, 1995.
98. PRAT, A G., I. L. REISIN, D. A. AUSIELLO, AND H. F. CANTIELLO. Cellular ATP release by the cystic fibrosis transmembrane conductance regulator. Am. J. Physiol. 270 (Cell Physiol. 39): C538-C545, 1996.
99. + channels in the kidney. Naunyn-Schmiedbergs Arch. Pharmacol, 354: 213-225, 1996.
100. QUINTON, P. M. Chloride impermeability in cystic fibrosis. Nature 301: 421-422, 1983.
101. 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.
102. 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. Cystic fibrosis transmembrane conductance regulator and adenosine triphosphate. Science 275: 1325, 1997.
103. 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.
104. RIORDAN, J. R., J. M. ROMMENS, B. 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.
105. ROMMENS, J. M., M. C. IANNUZZI, B.-S. KEREM, M. L. DRUMM, G. MELMER, M. DEAN, R. ROZMAHEL, J. L. COLE, D. KENNEDY, N. HIDAKA, M. ZSIGA, M. BUCHWALD, J. R. RIORDAN, L.-C. TSUI, AND F. S. COLLINS. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 245: 1059-1065, 1989.
106. 2+ channels by G-protein-coupled receptors. FASEB J. 2: 2784-2790, 1988.
107. ROZMAHEL, R., M. WILSCHANSKI, A. MATIN, S. PLYTE, M. OLIVER, W. AUERBACH, A. MOORE, J. FORSTNER, P. DURIE, J. NADEAU, C. BEAR, AND L. C. TSUI. Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor. Nature Genet. 12: 280-287, 1996.
108. ATP, channel (Kir 1.1-CFTR) (Abstract). J. Am. Soc. Nephrol. 7: 1289, 1996.
109. 1 adenosine receptor agonists mobilize intracellular calcium and activate potassium and chloride currents in normal and cystic fibrosis airway epithelial cells. J. Biol. Chem. 268: 24779-24784, 1993.
110. - channels. J. Biol. Chem. 271: 10210-10216, 1996.
111. SANSOM, S. C., AND G. H. GIEBISCH. Potassium homeostasis. In: Textbook of Nephrology. Baltimore, MD: Williams & Wilkins, 1995, p. 293-305.
112. + channels in the cortical collecting duct by AVP and mineralocorticoids. Kidney Int. 41: 255-268, 1992.
113. SCHOUMACHER, R. A., R. L. SHOEMAKER, D. R. HALM, E. A. TALLANT, R. W. WALLACE, AND R. A. FRIZZELL. Phosphorylation fails to activate chloride channels from cystic fibrosis airway cells. Nature 330: 752-754, 1987.
114. SCHWIEBERT, E. M., M. E. EGAN, AND W. B. GUGGINO. Assays to study the dynamics, mechanisms, and regulation of ATP release from epithelial and non-epithelial cells. Methods Enzymol. 292: 664-675, 1998.
115. 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.
116. SCHWIEBERT, E. M., T. FLOTTE, G. R. CUTTING, AND W. B. GUGGINO. Both CFTR and outwardly rectifying chloride channels contribute to cyclic AMP-stimulated whole cell chloride currents. Am. J. Physiol 266 (Cell Physiol. 35): C1464-C1477, 1994.
117. SCHWIEBERT, E. M., K. H. KARLSON, P. A. FRIEDMAN, P. DIETL, W. S. SPIELMAN, AND B. A. STANTON. Adenosine regulates a chloride channel via protein kinase C and a G protein in a rabbit cortical collecting duct cell line. J. Clin. Invest. 89: 834-841, 1992.
118. SCHWIEBERT, E. M. M. M. MORALES, S. DEVIDAS, M. E. EGAN, AND W. B. GUGGINO. Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmenibrane conductance regulator. Proc. Natl. Acad. Sci. USA 95: 2674-2679, 1998.
119. SHEPPARD, D. N., D.P. RICH, L. S. OSTEDGAARD, R. J. GREGORY, A. E. SMITH, AND M. J. WELSH. Mutations in CFTR associated with mild disease form Cl channels with altered pore properties. Nature 362: 160-164, 1993.
120. SHEPPARD, D. N., AND M. J. WELSH. Effect of ATP-sensitive channel regulators on CFTR currents. J. Gen. Physiol. 100: 573-591, 1992.
121. SHORT, D. B., K. W. TROTTER, D. RECZEK, S. M. KREDA, A. BRETSCHER, R. C. BOUCHER, M. J. STUTTS, AND S. L. MILGRAM. An apical PDZ protein anchors the cystic fibrosis transmembrane conductance regulator to the cytoskeleton. J. Biol. Chem. 273: 19797-19801, 1998.
122. STUTTS, M. J. Regulation of other airway epithelial ion channels by CFTR. In: Current Topics in Cystic Fibrosis, edited by J. A. Dodge, D. J. H. Brock, and J. H. Widdicombe. New York: Dekker, 1997.
123. STUTTS, M. J., C. M. CANESSA, J. C. OLSEN, M. HAMRICK, J. A. COHN, B. C. ROSSIER, AND R. C. BOUCHER. CFTR as a cAMP-dependent regulator of sodium channels. Science 269: 847-850, 1995.
124. - channels in normal and cystic fibrosis airway epithelial cells by extracellular ATP. Proc. Natl. Acad. Sci. USA 89: 1621-1625, 1992.
125. STUTTS, M. J., J. G. FITZ, A. M. PARADISO, AND R. C. BOUCHER. Multiple modes of regulation of airway epithelial chloride secretion by extracellular ATP. Am. J. Physiol. 267 (Cell Physiol. 36): C1442-C1451, 1994.
126. STUTTS, M. J., B. C. ROSSIER, AND R. C. BOUCHER. CFTR inverts PKA-mediated regulation of ENaC single channel kinetics. J. Biol. Chem. 272: 14037-14040, 1997.
127. SUGITA, M., Y. YUE, AND J. K. FOSKETT. CFTR Cl channel and CFTR-associated ATP channel: distinct pores regulated by common gates. EMBO J. 17: 898-908, 1998.
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. 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.
130. TODD-TURLA, K., E. RUSVAI, A. NARAY-FEJES-TOTH, AND G. FEJES-TOTH. CFTR expression in cortical collecting duct cells. Am. J. Physiol. 270 (Renal Fluid Electrolyte Physiol. 39): F237-P244, 1996.
131. VALVERDE, M. A., M. DIAZ, F. V. SEPULVEDA, D. R. GILL, S. C. HYDE, AND C. F. HIGGINS. Volume-regulated chloride conductance associated with the human multidrug-resistance P-glycoprotein. Nature 355: 830-833, 1992.
132. VALVERDE, M. A., J. A. O'BRIEN, F. V. SEPULVEDA, R. A. RAT-CLIFF, M. J. EVANS, AND W. H. COLLEDGE. Impaired cell volume regulation in intestinal crypt epithelia of cystic fibrosis mice. Proc. Natl. Acad. Sci. USA 92: 9038-9041, 1995.
133. VAN HELVOORT, A., A. J. SMITH, H. SPRONG, I. FRITZSCHE, A. H. SCHINKEL, P. BORST, AND G. VAN MEER. mdr1 P-glycoprotein is a lipid translocase of broad specificity, while mdr3 P-glycoprotein specifically translocates phosphatidylcholine. Cell 87: 507-517, 1996.
134. WANG, S., R. W. RAAB, P. J. SCHATZ, W. B. GUGGINO, AND M. LI. Peptide binding consensus of the NHE-RF-PDZ1 domain matches the C-terminal sequence of the cystic fibrosis transmembrane conductance regulator (CFTR). FEBS Lett. 427: 103-108, 1998.
135. + channel in the apical membrane of rabbit thick ascending limb of Henle's loop. Am. J. Physiol. 258 (Renal Fluid Electrolyte Physiol. 27): F244-F253, 1990.
136. WELSH, M. J., AND A. E. SMITH. Molecular mechanisms of CFTR chloride channel dysfunction in CF. Cell 73: 1251-1254, 1993.
137. 2+ ionophores in cystic fibrosis airway epithelia. Am. J. Physiol. 256 (Cell Physiol. 25): C226-C233, 1989.
138. WILLUMSEN, N. J., AND R. C. BOUCHER. Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium. Am. J. Physiol, 261 (Cell Physiol, 30): C332-C341, 1991.
139. YUE, G., W. J. RUSSELL, D. J. BENOS, R. M. JACKSON, M. A. OLMAN, AND S. MATALON. Increased expression and activity of sodium channels in alveolar type II cells of hyperoxic rats. Proc. Natl. Acad. Sci. USA 92: 8418-8422, 1995.
140. + channel. Am. J. Physiol. 266 (Cell Physiol 35): C809-C824, 1994.