Molecular physiology of voltage-gated chloride channels

Physiological Reviews

Volumn 74, Issue 4, 1994, Pages 813-827

  Pusch, Michael ^a   Jentsch, Thomas J ^a  

^a UNIVERSITY OF HAMBURG   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE CHANNEL; CHLORIDE ION; COMPLEMENTARY DNA; CYCLIC AMP DEPENDENT PROTEIN KINASE; GLYCOPROTEIN P; MEMBRANE PROTEIN; MESSENGER RNA; PHOSPHOLEMMAN; TRANSMEMBRANE CONDUCTANCE REGULATOR; UNCLASSIFIED DRUG;

CHANNEL GATING; CHLORIDE CONDUCTANCE; CHROMOSOME 7Q; ELECTRIC ORGAN; GENETIC LINKAGE; HUMAN; KIDNEY; MEMBRANE POTENTIAL; MYOTONIA; NONHUMAN; OOCYTE; POINT MUTATION; PRIORITY JOURNAL; REVIEW; SKELETAL MUSCLE; THOMSEN DISEASE; TORPEDO; XENOPUS;

EID: 0027996879     PISSN: 00319333     EISSN: None     Source Type: Journal    
DOI: 10.1152/physrev.1994.74.4.813     Document Type: Review

References (191)

1. ABDALLA, J. A., W. L. CASLEY, H. K. COUSIN, A. J. HUDSON, A. J. MURPHY, F. C. CORNÉLIS, L. HASHIMOTO, AND G. C. EBERS. Linkage of Thomsen disease to the T-cell receptor beta (TCRB) locus on chromosome 7q35. Am. J. Hum. Genet. 51: 579-584, 1992.
2. ABE, T., K. TAKEUCHI, K. ISHII, AND K. ABE. Molecular cloning and expression of a rat cDNA encoding MDCK-type chloride channel. Biochim. Biophys. Acta 1173: 353-356, 1993.
3. ACKERMAN, M. J., AND D. E. CLAPHAM. Cardiac chloride channels. Trends Cardiovasc. Med. 3: 23-28, 1993.
4. ACKERMAN, S. J., G. C. KOWDLEY, L. R. JONES, G. SZABO, AND J. R. MOORMAN. Voltage-dependent ion selectivity of phospholemman channels in planar lipid bilayers (Abstract). Biophys. J. 66: A216, 1994.
5. ACKERMAN, M. J., K. D. WICKMAN, AND D. E. CLAPHAM. Hypotonicity activates a chloride current in Xenopus oocytes. J. Gen. Physiol. 103: 153-179, 1994.
6. AL-AWQATI, Q., J. BARASCH, AND D. W. LANDRY. Proton pumps and chloride channels in secretory vesicles. In: Secretion and Its Control, edited by G. S. Oxford and C. M. Armstrong. New York: Rockefeller Univ. Press, 1989, p. 283-294.
7. - currents are activated by cell swelling but do not contribute to cell volume regulation. Cancer Res. 54: 618-622, 1994.
8. ALTENBERG, G. A., C. G. VANOYE, E. S. HAN, J. D. DEITMER, AND L. REUSS. Relationships between rhodamine 123 transport, cell volume, and ion-channel function of P-glycoprotein. J. Biol. Chem. 269: 7145-7149, 1994.
9. 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.
10. 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 Wash. DC 253: 202-205, 1991.
11. ARMSTRONG, C. M., AND F. J. BEZANILLA. Inactivation of the sodium channel. II. Gating current experiments. J. Gen. Physiol. 70: 567-590, 1977.
12. - channels. Nature Lond. 365: 850-852, 1993.
13. BAHINSKI, A., A. C. NAIRN, P. GREENGARD, AND D. C. GADSBY. Chloride conductance regulated by cyclic AMP-dependent protein kinase in cardiac myocytes. Nature Lond. 340: 718-721, 1989.
14. A receptor: the receptor/channel superfamily. Trends Neurosci. 10: 502-509, 1987.
15. BAUER, C. K., K. STEINMEYER, J. R. SCHWARZ, AND T. J. JENTSCH. Completely functional double-barreled chloride channel expressed from a single Torpedo cDNA. Proc. Natl. Acad. Sci. USA 88: 11052-11056, 1991.
16. - channel gating to an ATP hydrolysis cycle. Neuron 12: 473-482, 1994.
17. - channel activity in Xenopus oocytes expressing the cystic fibrosis gene. J. Biol. Chem. 266: 19142-19145, 1991.
18. 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. Cell 68: 809-818, 1992.
19. BECKER, P. E. Myotonia Congenita and Syndromes Associated With Myotonia. Clinical-Genetic Studies of the Non-Dystrophic Myotonias. Stuttgart, Germany: Thieme, 1977.
20. BECKER, P. E. Heterozygote manifestation in recessive generalized myotonia. Hum. Genet. 46: 325-329, 1979.
21. BETZ, H. Structure and function of inhibitory glycine receptors. Q. Rev. Biophys. 25: 381-394, 1992.
22. BLACHLY-DYSON, E., S. PENG, M. COLOMBINI, AND M. FORTE. Selectivity changes in site-directed mutants of the VDAC ion channel: structural implications. Science Wash. DC 247: 1233-1236, 1990.
23. BLATZ, A. L. Chloride channels in skeletal muscle. In: Chloride Channels and Carriers in Nerve, Muscle, and Glial Cells, edited by F. J. Alvarez-Leefmans and J. M. Russell. New York: Plenum, 1990, chapt. 16, p. 407-420.
24. BLATZ, A. L., AND K. L. MAGLEBY. Single chloride-selective channels at resting membrane potentials in cultured rat skeletal muscle. Biophys. J. 47: 119-123, 1985.
25. BRADBURY, N. A., T. JILLING, G. BERTA, R. J. SORSCHER, R. J. BRIDGES, AND K. L. KIRK. Regulation of plasma membrane recycling by CFTR. Science Wash. DC 256: 530-532, 1992.
26. BRETAG, A. H. Muscle chloride channels. Physiol. Rev. 67: 618-724, 1987.
27. BREUER, W. Reconstitution of a kidney chloride channel and its identification by covalent labelling. Biochim. Biophys. Acta 1022: 229-236, 1990.
28. BREUER, W., I. N. SLOTKI, D. A. AUSIELLO, AND I. Z. CABANTCHIK. Induction of multidrug resistance downregulates the expression of CFTR in colon epithelial cells. Am. J. Physiol. 265 (Cell Physiol. 34): C1711-C1715, 1993.
29. BRYANT, S. H., AND A. MORALES-AGUILERA. Chloride conductance in normal and myotonic muscle fibres and the action of monocarboxylic aromatic acids. J. Physiol. Lond. 219: 367-383, 1971.
30. CALA, P. M. Principles of cell volume regulation: ion flux pathways and the roles of anions. In: Chloride Channels and Carriers in Nerve, Muscle, and Glial Cells, edited by F. J. Alvarez-Leefmans and J. M. Russell. New York: Plenum, 1990, chapt. 2, p. 67-83.
31. CAMERINO, D., AND S. H. BRYANT. Effects of denervation and colchicine treatment on the chloride conductance of rat skeletal muscle fibers. J. Neurobiol. 7: 221-228, 1976.
32. CANTIELLO, H. F., A. G. PRATT, J. V. BONVENTRE, C. C. CUNNINGHAM, J. H. HARTWIG, AND D. A. AUSIELLO. Actin-binding protein contributes to cell volume regulatory ion channel activation in melanoma cells. J. Biol. Chem. 268: 4596-4599, 1993.
33. CHAMPIGNY, G., B. VERRIER, C. GÉRARD, J. MAUCHAMP, AND M. LADZUNSKI. Small conductance chloride channels in the apical membrane of thyroid cells. FEBS Lett. 259: 263-268, 1990.
34. CHEN, C.-J., J. E. CHIN, K. UEDA, D. P. CLARK, I. PASTAN, M. M. GOTTESMAN, AND I. B. RONINSON. Internal duplication and homology with bacterial transport proteins in the mdr1 (P-glycoprotein) gene from multidrug-resistant human cells. Cell 47: 381-389, 1986.
35. - channels in aplysia neurons. In: Chloride Channels and Carriers in Nerve, Muscle, and Glial Cells, edited by F. J. Alvarez-Leefmans and J. M. Russell. New York: Plenum, 1990, chapt. 14, p. 367-382.
36. CHUA, M., AND W. J. BETZ. Characterization of ion channels on the surface membrane of adult rat skeletal muscle. Biophys. J. 59: 1251-1260, 1991.
37. COLLINS, J. H., AND J. LESZYK. The "γ-subunit" of Na-K-AT-Pase: a small, amphiphilic protein with a unique amino acid sequence. Biochemistry 26: 8665-8668, 1987.
38. CONTE CAMERINO, D., A. DE LUCA, M. MAMBRINI, AND G. VRBOVÀ. Membrane ionic conductances in normal and denervated skeletal muscle of the rat during development. Pfluegers Arch. 413: 568-570, 1989.
39. CORNET, M., J. UBL, AND H.-A. KOLB. Cytoskeleton and ion movements during volume regulation in cultured PC12 cells. J. Membr. Biol. 133: 161-170, 1993.
40. CRAWFORD, I., P. C. MALONEY, P. 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.
41. DHALLAN, R. S., K.-W. YAU, K. A. SCHRADER, AND R. R. REED. Primary structure and functional expression of a cyclic nucleotide-activated channel from olfactory neurons. Nature Lond. 347: 184-187, 1990.
42. - channels in the rat colonic epithelium during regulatory volume decrease. Pfluegers Arch. 421: 530-538, 1992.
43. - concentration in the intralobular duct cells of mouse mandibular glands. J. Membr. Biol. 135: 289-295, 1993.
44. DOROSHENKO, P. Second messengers mediating activation of chloride current by intracellular GTPγS in bovine chromaffin cells. J. Physiol. Lond. 436: 725-738, 1991.
45. DOROSHENKO, P., R. PENNER, AND E. NEHER. Novel chloride conductance in the membrane of bovine chromaffin cells activated by intracellular GTPγS. J. Physiol. Lond. 436: 711-724, 1991.
46. DUSZYK, M., A. S. FRENCH, AND S. F. P. MAN. Noise analysis and single-channel observations of 4 pS channels in human airway epithelia. Biophys. J. 61: 583-587, 1992.
47. - channels by protein kinase A corrected by insertion of CFTR. Nature Lond. 358: 581-584, 1992.
48. EHRING, G. R., Y. V. OSIPCHUK, AND M. D. CAHALAN. Volume-activated chloride channels in drug-sensitive and -resistant cell lines (Abstract). Biophys. J. 66: A142, 1994.
49. EVANS, M. G., AND A. MARTY. Calcium-dependent chloride currents in isolated cells from rat lacrimal glands. J. Physiol. Lond. 378: 437-460, 1986.
50. FAHLKE, C., E. ZACHAR, U. HÄUSSLER, AND R. RÜDEL. Chloride channels in human skeletal muscle are regulated by G proteins. Pfluegers Arch. 421: 566-571, 1992.
51. FAHLKE, C., E. ZACHAR, AND R. RÜDEL. Single-channel recordings of chloride currents in cultured human skeletal muscle. Pfluegers Arch. 421: 108-116, 1992.
52. FAHLKE, C., E. ZACHAR, AND R. RÜDEL. Chloride channels with reduced single-channel conductance in recessive myotonia congenita. Neuron 10: 225-232, 1993.
53. --channel. Pfluegers Arch. 422: 159-167, 1992.
54. FOJO, A. T., K. UEDA, D. J. SLAMON, D. G. POPLACK, M. M. GOTTESMAN, AND I. PASTAN. Expression of a multidrug-resistance gene in human tumors and tissues. Proc. Natl. Acad. Sci. USA 84: 265-269, 1987.
55. - conductance cause hyperexcitability in recessive generalized myotonia (Becker). Muscle Nerve 14: 762-770, 1991.
56. FRIZZEL, R. A. The molecular physiology of cystic fibrosis. News Physiol. Sci. 8: 117-120, 1993.
57. FULLER, C. M., AND D. J. BENOS. CFTR! Am. J. Physiol. 263 (Cell Physiol. 32): C267-C286, 1992.
58. 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 Lond. 363: 263-266, 1993.
59. GABRIEL, S. E., AND G. W. FORSYSTH. Candidate proteins for conductive chloride transport in porcine ileal brush-border membrane. J. Biol. Chem. 266: 17764-17769, 1991.
60. GEORGE, A. L., JR., M. A. CRACKOWER, J. A. ABDALLA, A. J. HUDSON, AND G. C. EBERS. Molecular basis of Thomsen's disease (autosomal dominant myotonia congenita). Nature Genet. 3: 305-310, 1993.
61. - pump? Am. J. Physiol. 255 (Regulatory Integrative Comp. Physiol. 24): R677-R692, 1988.
62. GILL, D. R., S. C. HYDE, C. F. HIGGINS, M. A. VALVERDE, G. M. MINTENIG, AND F. V. SEPÚLVEDA. Separation of drug transport and chloride channel functions of the human multi-drug resistance P-glycoprotein. Cell 71: 23-32, 1992.
63. GOLDBERG, A. F. X., AND C. MILLER. Solubilization and functional reconstitution of a chloride channel from Torpedo californica electroplax. J. Membr. Biol. 124: 199-206, 1991.
64. GREGER, R., M. BLEICH, AND E. SCHLATTER. Ion channels in the thick ascending limb of Henle's loop. Renal Physiol. Biochem. 13: 37-50, 1990.
65. GRENNINGLOH, G., A. RIENITZ, B. SCHMITT, C. METHFESSEL, M. ZENSEN, K. BEYREUTHER, E. D. GUNDELFINGER, AND H. BETZ. The strychnine-binding subunit of the glycine receptor shows homology with nicotinic acetylcholine receptors. Nature Lond. 328: 215-220, 1987.
66. GRONEMEIER, M., A. CONDIE, J. PROSSER, K. STEINMEYER, T. J. JENTSCH, AND H. JOCKUSCH. Nonsense and missense mutations in the muscular chloride channel gene Clc-1 in mytotonic mice. J. Biol. Chem. 269: 5963-5967, 1994.
67. GROS, P., B. BENNERIAH, J. M. CROOP, AND D. E. HOUSMAN. Isolation and expression of a complementary DNA that confers multidrug resistance. Nature Lond. 323: 728-731, 1986.
68. GROS, P., J. CROOP, AND D. E. HOUSMAN. Mammalian multi-drug resistance gene: complete cDNA sequence indicating strong homology to bacterial transport proteins. Cell 47: 371-380, 1986.
69. GRÜNDER, S., A. THIEMANN, M. PUSCH, AND T. J. JENTSCH. Regions involved in the opening of ClC-2 chloride channel by voltage and cell volume. Nature Lond. 360: 759-763, 1992.
70. HAMILL, O. P., A. MARTY, E. NEHER, B. SAKMANN, AND F. J. SIGWORTH. Improved patch-clamp techniques for high resolution current recording from cells and cell-free membrane patches. Pfluegers Arch. 391: 85-100, 1981.
71. - currents acivated by cell swelling (Abstract). Biophys. J. 66: A99, 1994.
72. HANKE, W., AND C. MILLER. Single chloride channels from Torpedo electroplax. Activation by protons. J. Gen. Physiol. 82: 25-42, 1983.
73. HEINY, J. A., J. R. VALLE, AND S. H. BRYANT. Optical evidence for a chloride conductance in the T-system of frog skeletal muscle. Pfluegers Arch. 416: 288-295, 1990.
74. HIGGINS, C. F. ABC transporters: from microorganisms to man. Annu. Rev. Cell. Biol. 8: 67-113, 1992.
75. HILLE, B. Ionic Channels of Excitable Membranes. Sunderland, MA: Sinauer, 1984.
76. HOFFMANN, E. K., AND L. O. SIMONSON. Membrane mechanisms in volume and pH regulation in vertebrate cells. Physiol. Rev. 69: 315-382, 1989.
77. HOFFMANN, E. K., AND H. H. USSING. Membrane mechanisms in volume regulation in vertebrate cells and epithelia. In: Membrane Transport in Biology, edited by J. A. Schafer, H. H. Ussing, P. Kristensen, and G. H. Giebisch. Berlin: Springer, 1992, vol. 5.
78. HOSHI, T., W. N. ZAGOTTA, AND R. W. ALDRICH. Biophysical and molecular mechanisms of Shaker potassium channel inactivation. Science Wash. DC 250: 533-538, 1990.
79. HWANG, T.-C., S. E. GUGGINO, AND W. B. GUGGINO. Direct modulation of secretory chloride channels by arachidonic and other cis unsaturated fatty acids. Proc. Natl. Acad. Sci. USA 87: 5706-5709, 1990.
80. IAIZZO, P. A., C. FRANKE, H. HATT, W. SPITTELMEISTER, K. RICKER, R. RUDEL, AND F. LEHMANN-HORN. Altered sodium channel behaviour causes myotonia in dominantly inherited myotonia congenita. Neuromuscular Disorders 1: 47-53, 1991.
81. ISHIBASHI, K., S. SASAKI, S. UCHIDA, T. IMAI, AND F. MARUMO. Tissue expression of mRNA of chloride channel from MDCK cells and its regulation by protein kinases. Biochem. Biophys. Res. Commun. 192: 561-567, 1993.
82. JENTSCH, T. J., A. M. GARCIA, AND H. F. LODISH. Primary structure of a novel 4-acetamido-4′-isothiocyanostilbene-2,2′-disulphonic acid (SITS)-binding membrane protein highly expressed in Torpedo californica electroplax. Biochem. J. 261: 155-166, 1989.
83. JENTSCH, T. J., M. PUSCH, A. REHFELDT, AND K. STEINMEYER. The ClC family of voltage-gated chloride channels: structure and function. Ann. NY Acad. Sci. 707: 285-293, 1993.
84. JENTSCH, T. J., K. STEINMEYER, AND G. SCHWARZ. Primary structure of Torpedo marmorata chloride channel isolated by expression cloning in Xenopus oocytes. Nature Lond. 348: 510-514, 1990.
85. JOKUSCH, H., M. GRONEMEIER, A. CONDIE, J. PROSSER, T. J. JENTSCH, K. STEINMEYER, AND R. KLOCKE. Molecular physiology of myotonia: defects of the muscular chloride channel gene Clc-1 and activity dependent regulation of its expression (Abstract). J. Cell. Biochem. 18, Suppl. D: 528, 1994.
86. JOCKUSCH, H., J. REININGHAUS, I. STUHLFAUTH, AND M. ZIPPEL. Reduction of myosin-light-chain phosphorylation and of parvalbumin content in myotonic mouse muscle and its reversal by tocainide. Eur. J. Biochem. 171: 101-105, 1988.
87. KAUPP, U. B., T. NIIDOME, T. TANABE, S. TERADA, W. BÖNIGK, W. STÜHMER, N. J. COOK, K. KANGAWA, H. MATSUO, T. HIROSE, T. MIYATA, AND S. NUMA. Primary structure and functional expression from complementary DNA of the rod photoreceptor cyclic GMP-gated channel. Nature Lond. 342: 762-766, 1989.
88. KERSTING, U., S. NAPATHORN, AND K. R. SPRING. Necturus gallbladder epithelial cell volume regulation and inhibitors of arachidonic acid metabolism. J. Membr. Biol. 135: 11-18, 1993.
89. KIEFERLE, S., P. FONG, M. BENS, A. VANDEWALLE, AND T. J. JENTSCH. Two highly homologous members of the ClC chloride channel family in both rat and human kidney. Proc. Natl. Acad. Sci. USA. In press.
90. KOCH, M. C., K. RICKER, M. OTTO, F. WOLF, B. ZOLL, C. LORENZ, K. STEINMEYER, AND T. J. JENTSCH. Evidence for genetic homogeneity in autosomal recessive generalized myotonia (Becker). J. Med. Genet. 30: 914-917, 1993.
91. KOCH, M. C., K. STEINMEYER, C. LORENZ, K. RICKER, F. WOLF, M. OTTO, B. ZOLL, F. LEHMANN-HORN, K.-H. GRZESCHIK, AND T. J. JENTSCH. The skeletal muscle chloride channel in dominant and recessive human myotonia. Science Wash. DC 257: 797-800, 1992.
92. KOWDLEY, G. C., S. J. ACKERMAN, J. E. JOHN, L. R. JONES, AND J. R. MOORMAN. Is phospholemman an ion channel, or does it modulate an endogenous hyperpolarization-activated chloride current in Xenopus oocytes? (Abstract). Biophys. J. 64: A95, 1993.
93. KOWDLEY, G. C., S. J. ACKERMAN, J. E. JOHN, L. R. JONES, AND J. R. MOORMAN. Hyperpolarization activated chloride currents in Xenopus oocytes. J. Gen. Physiol. 103: 217-230, 1994.
94. KOWDLEY, G. C., J. R. MOORMAN, S. J. ACKERMAN, G. SZABO, AND L. R. JONES. Recombinant phospholemman forms chloride channels in planar lipid bilayers (Abstract). Biophys. J. 66: A216, 1994.
95. Cln. Cell 76: 439-448, 1994.
96. KUBO, M., AND Y. OKADA. Volume regulatory CJ- channel currents in cultured human epithelial cells. J. Physiol. Lond. 456: 351-371, 1992.
97. 2+, hypotonie cell swelling and 8-Br-cGMP. Pfluegers Arch. 421: 238-246, 1992.
98. KWIECINSKI, H., F. LEHMANN-HORN, AND R. RÜDEL. Drug-induced myotonia in human intercostal muscle. Muscle Nerve 11: 576-581, 1988.
99. LAMBERT, I. A. Effect of arachidonic acid, fatty acids, prostaglandins, and leukotrienes on volume regulation in Ehrlich ascites tumor cells. J. Membr. Biol. 98: 207-221, 1987.
100. LAMBERT, I. A., E. K. HOFFMANN, AND P. CHRISTENSEN. Role of prostaglandins and leukotrienes in volume regulation by Ehrlich ascites tumor cells. J. Membr. Biol. 98: 247-256, 1987.
101. LANDRY, D. W., M. H. AKABAS, C. REDHEAD, A. EDELMAN, E. J. CRAGOE, AND Q. AL-AWQATI. Purification and reconstitution of chloride channels from kidney and trachea. Science Wash. DC 244: 1469-1472, 1989.
102. LANDRY, D., S. SULLIVAN, M. NICOLAIDES, C. REDHEAD, A. EDELMAN, M. FIELD, Q. AL-AWQATI, AND J. EDWARDS. Molecular cloning and characterization of p64, a chloride channel protein from kidney microsomes. J. Biol Chem. 268:14948-14955, 1993.
103. LÄUGER, P. Electrogenic Ion Pumps. Sunderland, MA: Sinauer, 1991.
104. LEWIS, R. S., P. E. ROSS, AND M. D. CAHALAN. Chloride channels activated by osmotic stress in T lymphocytes. J. Gen. Physiol. 101: 801-826, 1993.
105. 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 Lond. 331: 358-360, 1988.
106. LIPICKY, R. J., AND S. H. BRYANT. Sodium, potassium, and chloride fluxes in intercostal muscle from normal goats and goats with hereditary myotonia. J. Gen. Physiol. 50: 89-111, 1966.
107. LIPICKY, R. J., S. H. BRYANT, AND J. H. SALMON. Cable parameters, sodium, potassium, chloride, water content, and potassium efflux in isolated external intercostal muscle of normal volunteers and patients with myotonia congenita. J. Clin. Invest. 50: 2091-2103, 1971.
108. LORENZ, C., C. MEYER-KLEINE, K. STEINMEYER, M. C. KOCH, AND T. J. JENTSCH. Genomic organization of the human muscle chloride channel ClC-1 and analysis of novel mutations leading to Becker-type myotonia. Hum. MoL Genet. 3: 941-946, 1994.
109. 1C receptor by electrophysiological assays of mRNA-injected Xenopus ooyctes. Proc. Natl. Acad. Sci. USA 84: 4332-4336, 1987.
110. 1A receptors in CHO cells. Am. J. Physiol. 264 (Renal Fluid Electrolyte Physiol. 33): F490-F495, 1993.
111. MARTIN, D. K. Small conductance chloride channels in acinar cells from rat mandibular salivary gland are directly controlled by a G-protein. Biochem. Biophys. Res. Commun. 192: 1266-1273, 1993.
112. MARTY, A., Y. P. TAN, AND A. TRAUTMANN. Three types of calcium-dependent channels in rat lacrimal glands. J. Physiol. Lond. 377: 293-325, 1984.
113. - efflux. J. Membr. Biol. 136: 55-62, 1993.
114. MAYER, M. L., D. G. OWEN, AND J. L. BARKER. Calcium dependent chloride currents in vertebrate central neurons. In: Chloride Channels and Carriers in Nerve, Muscle, and Glial Cells, edited by F. J. Alvarez-Leefmans and J. M. Russell. New York: Plenum, 1990, chapt. 13, p. 355-364.
115. 84 intestinal epithelial layers. FEBS Lett. 304: 233-236, 1992.
116. MEHRKE, G., H. BRINKMEIER, AND H. JOCKUSCH. The myotonic mouse mutant adr: electrophysiology of the muscle fiber. Muscle Nerve 11: 440-446, 1988.
117. MERCER, R. W., D. BIEMESDERFER, D. P. BLISS, JR., J. H. COLLINS, AND B. FORBUSH III. Molecular cloning and immunological characterization of the γ polypeptide, a small protein associated with the Na,K-ATPase. J. Cell Biol. 121: 579-586, 1993.
118. MEYER-KLEINE, C., K. RICKER, M. OTTO, M. C. KOCH, K. STEINMEYER, AND T. J. JENTSCH. A recurrent 14 bp deletion in the CLCN1 gene associated with generalized myotonia (Becker). Hum. Mol. Genet. 3: 1015-1016, 1994.
119. MIDDLETON, R. E., D. J. PHEASANT, AND C. MILLER. Purification and functional reconstitution of a voltage-gated chloride channel from Torpedo electric organ (Abstract). Biophys. J. 66: A142, 1994.
120. MILLER, C. Open-state substructure of single chloride channels from Torpedo electroplax. Philos. Trans. R. Soc. Lond. B Biol. Sci. 299: 401-411, 1982.
121. MILLER, C., AND E. A. RICHARD. The voltage-dependent chloride channel of Torpedo electroplax. Intimations of molecular structure from quirks of single-channel function. In: Chloride Channels and Carriers in Nerve, Muscle, and Glial Cells, edited by F. J. Alvarez-Leefmans and J. M. Russell. New York: Plenum, 1990, p. 383-405.
122. MILLER, C., AND M. M. WHITE. Dimeric structure of single chloride channels from Torpedo electroplax. Proc. Natl. Acad. Sci. USA 81: 2772-2775, 1984.
123. MILLS, J. W., E. M. SCHWIEBERT, AND B. A. STANTON. Evidence for the role of actin filaments in regulating cell swelling. J. Exp. Zool. 268: 111-120, 1994.
124. MIMURA, C. S., S. R. HOLBROOK, AND G. F.-L. AMES. Structural model of the nucleotide-binding protein conserved component of periplasmic permeases. Proc. Natl. Acad. Sci. USA 88: 84-88, 1991.
125. MINTENING, G. M., M. A. VALVERDE, F. V. SEPÚLVEDA, D. R. GILL, S. C. HYDE, J. KIRK, AND C. F. HIGGINS. Specific inhibitors distinguish the chloride channel and drug transporter functions associated with the human multidrug resistance P-glycoprotein. Receptors Channels 1: 305-313, 1994.
126. MOORMAN, J. R., C. J. PALMER, J. E. JOHN III, M. E. DURIEUX, AND L. R. JONES. Phospholemman expression induces a hyperpolarization-activated chloride current in Xenopus oocytes. J. Biol. Chem. 267: 14551-14554, 1992.
127. MOUNSEY, J. P., L. T. HORNE, J. E. JOHN, K. P. LU, A. R. MEANS, L. R. JONES, AND J. R. MOORMAN. Co-injection of protein kinase A mRNA increases phospholemman chloride currents in Xenopus oocytes (Abstract). Biophys. J. 66: A216, 1994.
128. NISHIMOTO, I., J. A. WAGNER, H. SCHULMAN, AND P. GARDNER. Regulation of chloride channels by multifunctional CaM kinase. Neuron 6: 547-555, 1991.
129. NOULIN, J.-F., AND M. JOFFRE. Characterization and cyclic AMP-dependence of a hyperpolarization-activated chloride conductance in Leydig cells from mature rat testis. J. Membr. Biol. 133: 1-15, 1993.
130. A receptors. FASEB J. 4: 1469-1480, 1990.
131. PALADE, P. T., AND R. L. BARCHI. Characteristics of the chloride conductance in muscle fibers of the rat diaphragm. J. Gen. Physiol. 69: 325-342, 1977.
132. PALFREY, H. C., AND M. C. RAO. Na/K/Cl cotransport and its regulation. J. Exp. Biol. 106: 43-54, 1983.
133. PALMER, C. J., B. T. SCOTT, AND L. R. JONES. Purification and complete sequence determination of the major plasma membrane substrate for cAMP-dependent protein kinase and protein kinase C in myocardium. J. Biol. Chem. 266: 11126-11130, 1991.
134. PAULAIS, M., AND J. TEULON. cAMP-activated chloride channel in the basolateral membrane of the thick ascending limb of the mouse kidney. J. Membr. Biol. 113: 253-260, 1990.
135. PAULMICHL, M., M. GSCHWENTNER, E. WOLLE, A. SCHMARDA, M. RITTER, G. KANIN, H. ELLEMUNTER, W. WAITZ, AND P. DEETJEN. Insight into the structure-function relation of chloride channels. Cell Physiol. Biochem. 3: 374-387, 1993.
136. PAULMICHL, M., Y. LI, K. WICKMAN, M. ACKERMANN, E. PERALTA, AND D. E. CLAPHAM. New mammalian chloride channel identified by expression cloning. Nature Lond. 356: 238-241, 1992.
137. PENNER, R., G. MATTHEWS, AND E. NEHER. Regulation of calcium influx by second messengers in rat mast cells. Nature Lond. 334: 499-504, 1988.
138. PERES, A., AND G. BERNARDINI. A hyperpolarization-activated chloride current in Xenopus laevis oocytes under voltage-clamp. Pfluegers Arch. 399: 157-159, 1983.
139. 2+-activated cell volume recovery mechanisms. Annu. Rev. Physiol. 52: 27-42, 1990.
140. - conductance in a mouse neuroblastoma X rat dorsal root ganglion cell line (F11). Brain Res. 614: 178-184, 1993.
141. 3 transport in the proximal tubule. Am. J. Physiol. 256 (Renal Fluid Electrolyte Physiol. 25): F751-P756, 1989.
142. PUSCH, M., K. STEINMEYER, AND T. J. JENTSCH. Low single channel conductance of the major skeletal muscle chloride channel, CIC-1. Biophys. J. 66: 149-152, 1994.
143. RAN, S., AND D. J. BENOS. Isolation and functional reconstitution of a 38-kDa chloride channel from bovine tracheal membranes. J. Biol. Chem. 266: 4782-4788, 1991.
144. RAN, S., C. M. FULLER, M. P. ARRATE, R. LATORRE, AND D. J. BENOS. Functional reconstitution of a chloride channel protein from bovine trachea. J. Biol. Chem. 267: 20630-20637, 1992.
145. RASOLA, A., L. J. V. GALIETTA, D. C. GRUENERT, AND G. ROMEO. Ionic selectivity of volume-sensitive currents in human epithelial cells. Biochim. Biophys. Acta 1139: 319-323, 1992.
146. RASOLA, A., L. J. V. GALIETTA, D. C. GRUENERT, AND G. ROMEO. Volume-sensitive chloride currents in four epithelial cell lines are not directly correlated to the expression of the MDR-1 gene. J. Biol. Chem. 269: 1432-1436, 1994.
147. REDHEAD, C. R., A. E. EDELMAN, D. BROWN, D. W. LANDRY, AND Q. AL-AWQATI. A ubiquitous 64-kDa protein is a component of a chloride channel of plasma and intracellular membranes. Proc. Natl. Acad. Sci. USA 89: 3716-3720, 1992.
148. RICHARD, E. A., AND C. MILLER. Steady-state coupling of ion-channel conformations to a transmembrane ion gradient. Science Wash. DC 247: 1208-1210, 1990.
149. RIORDAN, J. R. The cystic fibrosis transmembrane conductance regulator. Annu. Rev. Physiol. 55: 609-630, 1993.
150. 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 Wash. DC 245: 1066-1073, 1989.
151. ROOS, A., AND W. F. BORON. Intracellular pH. Physiol. Rev. 61: 296-434, 1981.
152. RÜDEL, R. The myotonic mouse - a realistic model for the study of human recessive generalized myotonia. Trends Neurosci. 13: 1-3, 1990.
153. RÜDEL, R., K. RICKER, AND F. LEHMANN-HORN. Transient weakness and altered membrane characteristics in recessive generalized myotonia (Becker). Muscle Nerve 11: 202-211, 1988.
154. RUSSELL, J. M., AND W. F. BORON. Chloride transport in the squid giant axon. In: Chloride Channels and Carriers in Nerve, Muscle, and Glial Cells, edited by F. J. Alvarez-Leefmans and J. M. Russell. New York: Plenum, 1990, chapt. 3, p. 85-107.
155. 2 and inhibited by GTPγS. J. Physio. Lond. 461: 201-212, 1993.
156. A receptor shows a ligand-gated receptor super-family. Nature Lond. 328: 221-227, 1987.
157. 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 Lond. 330: 752-754, 1987.
158. SCHWIEBERT, E. M., J. W. MILLS, AND B. A. STANTON. Actin-based cytoskeleton regulates a chloride channel and cell volume in a renal cortical collecting duct cell line. J. Biol. Chem. 269: 7081-7089, 1994.
159. SOROTA, S. Swelling-induced chloride-sensitive current in canine atrial cells revealed by whole-cell patch-clamp. Circ. Res. 70: 679-687, 1992.
160. STEINMEYER, K., R. KLOCKE, C. ORTLAND, M. GRONEMEIER, H. JOCKUSCH, S. GRÜNDER, AND T. J. JENTSCH. Inactivation of muscle chloride channel by transposon insertion in myotonic mice. Nature Lond. 354: 304-308, 1991.
161. STEINMEYER, K., C. LORENZ, M. PUSCH, M. C. KOCH, AND T. J. JENTSCH. Multimeric structure of ClC-1 chloride channels as revealed by mutations in dominant myotonia congenita (Thomsen). EMBO J. 13: 737-743, 1994.
162. STEINMEYER, K., C. ORTLAND, AND T. J. JENTSCH. Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel. Nature Lond. 354: 301-304, 1991.
163. - currents in human neutrophils induced by cell swelling. Arn. J. Physiol. 265 (Cell Physiol. 34): C156-C165, 1993.
164. SUN, X. P., S. SUPPLISON, AND E. MAYER. Chloride channels in myocytes from rabbit colon are regulated by a toxin-sensitive G protein. Am. J. Physiol 264 (Gastrointest. Liver Physiol. 27): G774-G785, 1993.
165. TABARES, L., M. MAZZANTI, AND D. E. CLAPHAM. Chloride channels in the nuclear membrane. J. Membr. Biol. 123: 49-54, 1991.
166. 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 Lond. 366: 79-82, 1993.
167. TAGLIETTI, V., F. TANZI, R. ROMERO, AND L. SIMONCINI. Maturation involves suppression of voltage-gated currents in the frog oocyte. J. Cell. Physiol. 121: 576-588, 1984.
168. TAKUMI, T., H. OHKUBO, AND S. NAKANISHI. Cloning of a membrane protein that induces a slow voltage-gated potassium current. Science Wash. DC 242: 1042-1045, 1988.
169. TILLY, B. C., G. M. S. MANCINI, J. BIJMAN, P. G. M. VAN GAGELDONK, C. E. M. T. BEERENS, R. J. BRIDGES, H. R. DE JONGE, AND F. W. VERHEIJEN. Nucleotide-activated chloride channels in lysosomal membranes. Biochem. Biophys. Res. Commun. 187: 254-260, 1992.
170. TILLY, B. C., N. VAN DEN BERGHE, L. G. J. TERTOOLEN, M. C. EDIXHOVEN, AND H. R. DE JONGE. Protein phosphorylation is involved in osmoregulation of ionic conductances. J. Biol. Chem. 268:19919-19922, 1993.
171. THIEMANN, A., S. GRÜNDER, M. PUSCH, AND T. J. JENTSCH. A chloride channel widely expressed in epithelial and non-epithelial cells. Nature Lond. 356: 57-60, 1992.
172. THOMSEN, J. Tonische Krämpfe in willkürlich beweglichen Muskeln in Folge von ererbter psychischer Disposition. Arch. Psychiatr. Nervenkrankh. 6: 702-718, 1876.
173. TREZISE, A. E. O., P. R. ROMANO, D. R. GILL, S. C. HYDE, S. C. SEPÚLVEDA, M. BUCHWALD, AND C. F. HIGGINS. The multi-drug resistance and cystic fibrosis genes have complementary patterns of epithelial expression. EMBO J. 11: 4291-4303, 1992.
174. - channel. Am. J. Physiol. 262 (Cell Physiol. 31): C1056-C1068, 1992.
175. UCHIDA, S., S. SASAKI, T. FURUKAWA, M. HIRAOKA, T. IMAI, Y. HIRATA, AND F. MARUMO. Molecular cloning of a chloride channel that is regulated by
176. UEDA, K., M. CARDARELLI, M. M. GOTTESMAN, AND I. PASTAN. Expression of a full-length cDNA for the human MDR1 gene confers resistance to colchicine, doxorubicin and vinblastine. Proc. Natl. Acad. Sci. USA 84: 3004-3008, 1987.
177. VALVERDE, M. A., M. DÍAZ, F. V. SEPÚLVEDA, D. R. GILL, S. C. HYDE, AND C. F. HIGGINS. Volume-regulated chloride channels associated with the human multidrug-resistance P-glycoprotein. Nature Lond. 355: 830-833, 1992.
178. WANG, K., J. R. MOORMAN, J. E. JOHN, L. R. JONES, AND C. M. BAUMGARTEN. Phospholemman expression and phosphorylation by protein kinase A increase the volume of Xenopus oocytes (Abstract). Biophys. J. 66: A216, 1994.
179. WARNOCK, D. G., AND J. EVELOFF. K-Cl cotransport systems. Kidney Int. 36: 412-417, 1989.
180. WEBER-SCHÜRHOLZ, S., E. WISCHMEYER, M. LAURIEN, H. JOCKUSCH, T. SCHÜRHOLZ, D. W. LANDRY, AND Q. AL-AWQATI. Indanyloxyacetic acid-sensitive chloride channels from outer membranes of skeletal muscle. J. Biol. Chem. 268: 547-551, 1993.
181. WELSH, M. J., M. P. ANDERSON, D. P. RICH, H. A. BERGER, G. M. DENNING, L. S. OSTEGAARD, D. N. SHEPPARD, S. H. CHENG, R. J. GREGORY, AND A. E. SMITH. Cystic fibrosis transmembrane conductance regulator: a chloride channel with novel regulation. Neuron 8: 821-829, 1992.
182. WHITE, M. M., AND C. MILLER. A voltage-gated anion channel from the electric organ of Torpedo californica. J. Biol. Chem. 254: 10161-10166, 1979.
183. --channel from Torpedo electroplax. J. Gen. Physiol. 254: 10161-10166, 1981.
184. - or cAMP-dependent protein kinase. J. Membr. Biol. 122: 89-95, 1991.
185. - channels in basolateral renal medullary membranes. VII. Characterization of the intracellular anion binding site. J. Membr. Biol. 135: 145-152, 1993.
186. WISCHMEYER, E., E. NOLTE, R. KLOCKE, H. JOCKUSCH, AND H. BRINKMEIER. Development of electrical myotonia in the ADR mouse: role of chloride conductance in myotubes and neonate animals. Neuromuscular Disorders 3: 267-274, 1993.
187. WORRELL, R. T., A. G. BUTT, W. H. CLIFF, AND R. A. FRIZZELL. A volume-sensitive chloride conductance in human colonic cell line T84. Am. J. Physiol. 256 (Cell Physiol. 25): C1111-C1119, 1989.
188. WORRELL, R. T., AND R. A. FRIZZELL. CaMKII mediates stimulation of chloride conductance by calcium in T84 cells. Am. J. Physiol. 260 (Cell Physiol. 29): C877-C882, 1991.
189. YANTORNO, R. E., D. A. CARRE, M. COCA-PRADOS, T. KRUPIN, AND M. M. CIVAN. Whole cell patch clamping of ciliary epithelial cells during anisosmotic swelling. Am. J. Physiol. 262 (Cell Physiol. 31): C501-C509, 1992.
190. 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 Wash. DC 250: 568-571, 1990.
191. ZELLWEGER, H., L. PAVONE, A. BIONDI, V. CIMINO, F. GULETT, M. HART, V. IONESCU, F. MOLLICA, AND R. SCHIEKEN. Autosomal recessive generalized myotonia. Muscle Nerve 3: 176-180, 1980.