The CLC chloride channel family

Pflugers Archiv European Journal of Physiology

Volumn 437, Issue 6, 1999, Pages 783-795

  Jentsch, Thomas J ^a   Friedrich, Thomas ^a   Schriever, Antje ^a   Yamada, Hideomi ^a  

^a UNIVERSITY OF HAMBURG   (Germany)

Author keywords

Anion channel; Arabidopsis thaliana; CBS domain; Double barrel; Endocytosis; Gating; Human genetics; Kidney stones; Yeast gef1

Indexed keywords

CHLORIDE ION;

ACIDIFICATION; ANIMAL CELL; ARABIDOPSIS; CELL VOLUME; CHLORIDE TRANSPORT; EPITHELIUM CELL; MEMBRANE STRUCTURE; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; PROTEIN ASSEMBLY; REVIEW;

EID: 0032922760     PISSN: 00316768     EISSN: None     Source Type: Journal    
DOI: 10.1007/s004240050847     Document Type: Review

References (129)

1. 1. Adachi S, Uchida S, Ito H, Hata M, Hiroe M, Marumo F, Sasaki S (1994) Two isoforms of a chloride channel predominantly expressed in thick ascending limb of Henle's loop and collecting ducts of rat kidney. J Biol Chem 269:17677-17683
2. 2. Bateman A (1997) The structure of a domain common to archaebacteria and the homocystinuria disease protein. Trends Biochem Sci 22:12-13
3. 3. Bauer CK, Steinmeyer K, Schwarz JR, Jentsch TJ (1991) Completely functional double-barreled chloride channel expressed from a single Torpedo cDNA. Proc Natl Acad Sci USA 88:11052-11056
4. 4. Beck CL, Fahlke C, George AL Jr (1996) Molecular basis for decreased muscle chloride conductance in the myotonic goat. Proc Natl Acad Sci USA 93:11248-11252
5. 84 cells: outwardly vs. inwardly rectifying currents. J Physiol (Lond) 511: 45-54
6. 6. Borsani G, Rugarli EI, Taglialatela M, Wong C, Ballabio A (1995) Characterization of a human and murine gene (CLCN3) sharing similarities to voltage-gated chloride channels and to a yeast integral membrane protein. Genomics 7:131-141
7. 7. Brandt S, Jentsch TJ (1995) ClC-6 and ClC-7 are two novel broadly expressed members of the ClC family of chloride channels. FEBS Lett 377:15-20
8. 8. Bryant SH, Morales-Aguilera A (1971) Chloride conductance in normal and myotonic muscle fibres and the action of monocarboxylic aromatic acids. J Physiol (Lond) 219:367-383
9. Cln and ClC-6 in Xenopus oocytes induces an identical endogenous chloride conductance. J Biol Chem 272:3615-3621
10. 2+ pump SERCA2b. Biochem J 330:1015-1021
11. 11. Carew MA, Thorn P (1996) Identification of ClC-2-like chloride currents in pig pancreatic acinar cells. Pflügers Arch 433:84-90
12. 12. Chen TY (1998) Extracellular zinc ion inhibits ClC-0 chloride channels by facilitating slow gating. J Gen Physiol 112: 715-726
13. - channel. J Gen Physiol 108:237-250
14. 14. Chu S, Zeitlin PL (1997) Alternative mRNA splice variants of the rat ClC-2 chloride channel gene are expressed in lung: genomic sequence and organization of ClC-2. Nucleic Acids Res 25:4153-4159
15. 15. Chu S, Murray CB, Liu MM, Zeitlin PL (1996) A short ClC-2 mRNA transcript is produced by exon skipping. Nucleic Acids Res 24:3453-3457
16. 16. Clark S, Jordt SE, Jentsch TJ, Mathie A (1998) Characterization of the hyperpolarization-activated chloride current in dissociated rat sympathetic neurons. J Physiol (Lond) 506: 665-678
17. 17. Clayton GH, Staley KJ, Wilcox CL, Owens GC, Smith RL (1998) Developmental expression of ClC-2 in the rat nervous system. Brain Res Dev 108:307-318
18. 18. Conte Camerino D, De Luca A, Mambrini M, Vrbova G (1989) Membrane ionic conductances in normal and denervated skeletal muscle of the rat during development. Pflügers Arch 413:568-70
19. 19. Davis-Kaplan SR, Askwith CC, Bengtzen AC, Radisky D, Kaplan J (1998) Chloride is an allosteric effector of copper assembly for the yeast multicopper oxidase fet3p: an unexpected role for intracellular chloride channels. Proc Natl Acad Sci USA 95:13641-13645
20. 20. Devuyst O, Christie PT, Courtoy PJ, Beauwens R, Thakker RV (1999) Intra-renal and subcellular distribution of the human chloride channel, CLC-5, reveals a pathophysiological basis for Dent's disease. Hum Mol Genet 8:247-257
21. 21. Dinudom A, Komwatana P, Young JA, Cook DI (1995) A forskolin-activated Cl-current in mouse mandibular duct cells. Am J Physiol 268:G806-G812
22. 22. Duan D, Winter C, Cowley S, Hume JR, Horowitz B (1997) Molecular identification of a volume-regulated chloride channel. Nature 390:417-421
23. 23. Fahlke C, Zachar E, Rüdel R (1993) Chloride channels with reduced single-channel conductance in recessive Myotonia Congenita. Neuron 10:225-232
24. 24. Fahlke C, Rüdel R, Mitrovic N, Zhou M, George AL (1995) An aspartic acid residue important for voltage-dependent gating of human muscle chloride channels. Neuron 15:463-472
25. 25. Fahlke C, Rosenbohm A, Mitrovic N, George AL, Rüdel R (1996) Mechanisms of voltage-dependent gating in skeletal muscle chloride channels. Biophys J 71:695-706
26. 26. Fahlke C, Beck CL, George AL Jr (1997) A mutation in autosomal dominant myotonia congenita affects pore properties of the muscle chloride channel. Proc Natl Acad Sci USA 94:2729-2734
27. 27. Fahlke C, Durr C, George AL Jr (1997) Mechanism of ion permeation in skeletal muscle chloride channels. J Gen Physiol 110:551-564
28. 28. Fahlke C, Knittle T, Gurnett CA, Campbell KP, George AL Jr (1997) Subunit stoichiometry of human muscle chloride channels. J Gen Physiol 109:93-104
29. 29. Fahlke C, Yu HT, Beck CL, Rhodes TH, George AL Jr (1997) Pore-forming segments in voltage-gated chloride channels. Nature 390:529-532
30. 30. Fahlke C, Rhodes TH, Desai RR, George AL Jr (1998) Pore stoichiometry of a voltage-gated chloride channel. Nature 394:687-690
31. 31. Fisher SE, Black GC, Lloyd SE, Hatchwell E, Wrong O, Thakker RV, Craig IW (1994) Isolation and partial characterization of a chloride channel gene which is expressed in kidney and is a candidate for Dent's disease (an X-linked hereditary nephrolithiasis). Hum Mol Genet 3:2053-2059
32. - conductance cause hyperexcitability in recessive generalized myotonia (Becker). Muscle Nerve 14:762-770
33. 33. Friedrich T, Breiderhoff T, Jentsch TJ (1999) Mutational analysis demonstrates that ClC-4 and ClC-5 directly mediate plasma membrane currents. J Biol Chem 274:896-902
34. - current in human intestinal T84 epithelial cells by phosphorylation. J Physiol (Lond) 490:115-128
35. 35. Fritsch J, Edelman A (1997) Osmosensitivity of the hyperpolarization-activated chloride current in human intestinal T84 cells. Am J Physiol 272:C778-C786
36. 36. Fong P, Rehfeldt A, Jentsch TJ (1998) Determinants of slow gating in ClC-0, the voltage-gated chloride channel of Torpedo marmorata. Am J Physiol 274:C966-C973
37. 37. Furukawa T, Horikawa S, Terai T, Ogura T, Katayama Y, Hiraoka M (1995) Molecular cloning and characterization of a novel truncated from (ClC-2β) of ClC-2α (ClC-2G) in rabbit heart. FEBS Lett 375:56-62 [Erratum: FEBS Lett 403:111 (1997)]
38. 38. Furukawa T, Ogura T, Katayama Y, Hiraoka M (1998) Characteristics of rabbit ClC-2 current expressed in Xenopus oocytes and its contribution to volume regulation. Am J Physiol 274:C500-C512
39. 39. Gaxiola RA, Yuan DS, Klausner RD, Fink GR (1998) The yeast CLC chloride channel functions in cation homeostasis. Proc Natl Acad Sci USA 95:4046-4050
40. 40. George AL Jr, Crackower MA, Abdalla JA, Hudson AJ, Ebers GC (1993) Molecular basis of Thomsen's disease (autosomal dominant myotonia congenita). Nature Genet 3:305-310
41. 41. George AL Jr, Sloan-Brown K, Fenichel GM, Mitchell GA, Spiegel R, Pascuzzi RM (1994) Nonsense and missense mutations of the muscle chloride channel gene in patients with myotonia congenita. Hum Mol Genet 3:2071-2072
42. 42. Greene JR, Brown NH, DiDomenico BJ, Kaplan J, Eide DJ (1993) The GEF1 gene of Saccharomyces cerevisiae encodes an integral membrane protein; mutations in which have effects on respiration and iron-limited growth. Mol Gen Genet 241:542-553
43. 43. Gronemeier M, Condie A, Prosser J, Steinmeyer K, Jentsch TJ, Jockusch H (1994) Nonsense and missense mutations in the muscular chloride channel gene ClC-1 of myotonic mice. J Biol Chem 269:5963-5967
44. 44. Gründer S, Thiemann A, Pusch M, Jentsch TJ (1992) Regions involved in the opening of ClC-2 chloride channel by voltage and cell volume. Nature 360:759-762
45. 45. Gschwentner M, Nagl UO, Wöll E, Schmarda A, Ritter M, Paulmichl M (1995) Antisense oligonucleotides suppress cell-volume-induced activation of chloride channels. Pflügers Arch 430:464-470
46. 46. Günther W, Lüchow A, Cluzeaud F, Vandewalle A, Jentsch TJ (1998) ClC-5, the chloride channel mutated in Dent's disease, co-localizes with the proton pump in endocytotically active kidney cells. Proc Natl Acad Sci USA 95:8075-8080
47. 47. Gurnett CA, Kahl SD, Anderson RD, Campbell KP (1995) Absence of the skeletal muscle sarcolemma chloride channel ClC-1 in myotonic mice. J Biol Chem 270:9035-9038
48. 48. Hanke W, Miller C (1983) Single chloride channels from Torpedo electroplax. J Gen Physiol 82:25-45
49. 49. Hechenberger M, Schwappach B, Fischer WN, Frommer WB, Jentsch TJ, Steinmeyer K (1996) A family of putative chloride channels from Arabidopsis thaliana and functional complementation of a yeast strain with a CLC gene disruption. J Biol Chem 271:33632-33638
50. 50. Hryciw H Rychkov GY, Hughes BP, Bretag AH (1998) Relevance of the D13 region to the function of the skeletal muscle chloride channel, ClC-1. J Biol Chem 273:4304-4307
51. 51. Huber S, Schröppel B, Kretzler M, Schlöndorff D, Horster M (1998) Single cell RT-PCR analysis of ClC-2 mRNA expression in ureteric bud tip. Am J Physiol 274:F951-F957
52. pan disease. Kidney Int 54:1850-1856
53. 53. Jentsch T, Günther W (1997) Chloride channels: an emerging molecular picture. Bioessays 19:117-126
54. 54. Jentsch TJ, Steinmeyer K, Schwarz G (1990) Primary structure of Torpedo marmorata chloride channel isolated by expression cloning in Xenopus oocytes. Nature 348:510-514
55. 55. Jentsch TJ, Günther W, Pusch M, Schwappach B (1995) Properties of voltage-gated chloride channels of the ClC gene family. J Physiol (Lond) 482P:19S-26S
56. 56. Jordt SE, Jentsch TJ (1997) Molecular dissection of gating in the ClC-2 chloride channel. EMBO J 16:1582-1592
57. 57. Karolyi L, Koch MC, Grzeschik KH, Seyberth HW (1998) The molecular genetic approach to "Bartter's syndrome". J Mol Med 76:317-325
58. 58. Kawasaki M, Uchida S, Monkawa T, Miyawaki A, Mikoshiba K, Marumo F, Sasaki S (1994) Cloning and functional expression of a protein kinase C-regulated chloride channel abundantly expressed in rat brain neuronal cells. Neuron 12:597-604
59. 59. Kawasaki M, Suzuki M, Uchida S, Sasaki S, Marumo F (1995) Stable and functional expression of the ClC-3 chloride channel in somatic cell lines. Neuron 14:1285-1291
60. 60. Kieferle S, Fong P, Bens M, Vandewalle A, Jentsch TJ (1994) Two highly homologous members of the ClC chloride channel family in both rat and human kidney. Proc Natl Acad Sci USA 91:6943-6947
61. 61. Klocke R, Steinmeyer K, Jentsch TJ, Jockusch H (1994) Role of innervation, excitability, and myogenic factors in the expression of the muscular chloride channel ClC-1. A study on normal and myotonic muscle. J Biol Chem 269:27635-27639
62. 62. Koch MC, Steinmeyer K, Lorenz C, Ricker K, Wolf F, Otto M, Zoll B, Lehmann-Horn F, Grzeschik KH, Jentsch TJ (1992) The skeletal muscle chloride channel in dominant and recessive human myotonia. Science 257:797-800
63. 63. Koch MC, Ricker K, Otto M, Wolf F, Zoll B, Lorenz C, Steinmeyer K, Jentsch TJ (1993) Evidence for genetic homogeneity in autosomal recessive generalised myotonia (Becker). J Med Genet 30:914-917
64. 64. Koty PP, Pegoraro E, Hobson G, Marks HG, Turel A, Flagler D, Cadaldini M, Angelini C, Hoffman EP (1996) Myotonia and the muscle chloride channel: dominant mutations show variable penetrance and founder effect. Neurology 47:963-968
65. 65. Kubisch C, Schmidt-Rose T, Fontaine B, Bretag AH, Jentsch TJ (1998) ClC-1 chloride channel mutations in myotonia congenita: variable penetrance of mutations shifting the voltage-dependence. Hum Mol Genet 7:1753-1760
66. 66. Lehmann-Horn F, Mailander V, Heine R, George AL (1995) Myotonia levior is a chloride channel disorder. Hum Mol Genet 4:1397-1402
67. - channel from the renal cell line A6. Am J Physiol 273:C1176-C1185
68. 68. Lipicky RJ, Bryant H, Salmon JH (1971) Cable parameters, sodium, potassium, chloride, and water content, and potassium efflux in isolated external intercostal muscle of normal volunteers and patients with myotonia congenita. J Clin Invest 50:2091-2103
69. 69. Lloyd S, Pearce SHS, Fisher SE, Steinmeyer K, Schwappach B, Seinman SS, Harding B, Bolino M, Devoto M, Goodyer P, Regden SPA, Wrong O, Jentsch TJ, Craig IW, Thakker RV (1996) A common molecular basis for three inherited kidney stone diseases. Nature 379:445-449
70. 70. Lloyd SE, Günther W, Pearce SHS, Thomson A, Bianchi ML, Bosio M, Craig IW, Fisher SE, Scheinman SJ, Wrong O, Jentsch TJ, Thakker RV (1997) Characterisation of renal chloride channel CLCN5 mutations in hypercalciuric nephrolithiasis (kidney stone) disorders. Hum Mol Genet 6:1233-1239
71. 71. Lloyd SE, Pearce SHS, Günther W, Kawaguchi H, Igarashi T, Jentsch TJ, Thakker RV (1997) Idiopathic low molecular weight proteinuria associated with hypercalciuric nephrocalcinosis in Japanese children is due to mutations of the renal chloride channel (CLCN5). J Clin Invest 99:967-974
72. 72. Lorenz C, Meyer-Kleine C, Steinmeyer K, Koch MC, Jentsch TJ (1994) Genomic organization of the human muscle chloride channel CIC-1 and analysis of novel mutations leading to Becker-type myotonia. Hum Mol Genet 3:941-946
73. 73. Lorenz C, Pusch M, Jentsch TJ (1996) Heteromultimeric CLC chloride channels with novel properties. Proc Natl Acad Sci U SA 93:13362-13366
74. 74. Ludewig U, Pusch M, Jentsch TJ (1996) Two physically distinct pores in the dimeric ClC-0 chloride channel. Nature 383:340-343
75. 75. Ludewig U, Jentsch TJ, Pusch M (1997) Inward rectification in ClC-0 chloride channels caused by mutations in several protein regions. J Gen Physiol 10:165-171
76. 76. Ludewig U, Jentsch TJ, Pusch M (1997) Analysis of a protein region involved in permeation and gating of the voltage-gated Torpedo chloride channel ClC-0. J Physiol (Lond) 498:691-702
77. 77. Ludewig U, Pusch M, Jentsch TJ (1997) Independent gating of single pores in ClC-0 chloride channels. Biophys J 73:789-797
78. 78. Luyckx VA, Goda FO, Mount DB, Nishio T, Hall A, Hebert SC, Hammond TG, Yu AS (1998) Intrarenal and subcellular localization of rat CLC5. Am J Physiol 275:F761-F769
79. 79. Maduke M, Williams C, Miller C. (1998) Formation of CLC-0 chloride channels from separated transmembrane and cytoplasmic domains. Biochemistry 37:1315-1321
80. 80. Mailänder V, Heine R, Deymeer F, Lehmann-Horn F (1996) Novel muscle chloride channel mutations and their effects on heterozygous carriers. Am J Hum Genet 58:317-324
81. - channel. Am J Physiol 268:C191-C200
82. 82. Matsumura Y, Uchida S, Kondo Y, Miyazaki H, Ko SBH, Hayama A, Morimoto T, Liu W, Arisawa M, Sasaki S, Marumo F (1999) Overt nephrogenic diabetes insipidus in mice lacking the CLC-K1 chloride channel. Nature Genet 21:95-98
83. 83. Mehrke G, Brinkmeier H, Jockusch H (1988) The myotonic mouse mutant ADR: electrophysiology of the muscle fiber. Muscle & Nerve 11:440-446
84. 84. Middleton R, Pheasant DJ, Miller C (1994) Purification, reconstitution, and subunit composition of a voltage-gated chloride channel from Torpedo electroplax. Biochemistry 33: 13189-13198
85. 85. Middleton RE, Pheasant DJ, Miller C (1996) Homodimeric architecture of a ClC-type chloride ion channel. Nature 383:337-340
86. 86. Morimoto T, Uchida S, Sakamoto H, Kondo Y, Hanamizu H, Fukui M, Tomino Y, Nagano N, Sasaki S, Marumo F (1998) Mutations in CLCN5 chloride channel in Japanese patients with low molecular weight proteinuria. J Am Soc Nephrol 9:811-818
87. 87. Mukherjee S, Ghosh RN, Maxfield FR (1997) Endocytosis. Physiol Rev 77:759-803
88. 88. Murray CB, Morales MM, Flotte TR, McGrath-Morrow SA, Guggino WB, Zeitlin PL (1995) ClC-2: A developmentally dependent chloride channel expressed in the fetal lung and downregulated after birth. Am J Respir Cell Mol Biol 12:597-604
89. 89. Murray CB, Chu S, Zeitlin PL (1996) Gestational and tissue-specific regulation of ClC-2 chloride channel expression. Am J Physiol 271:L829-L837
90. 90. Obermüller N, Gretz N, Kriz W, Reilly RF, Witzgall R (1998) The swelling-activated chloride channel ClC-2, the chloride channel ClC-3, and ClC-5, a chloride channel mutated in kidney stone disease, are expressed in distinct subpopulations of renal epethelial cells. J Clin Invest 101:635-6421
91. - channels in rat parotid acinar cells with ClC-2 in a mammalian expression system. J Membr Biol 163:87-95
92. 92. Plassart-Schiess E, Gervais A, Eymard B, Lagueny A, Pouget J, Warter JM, Fardeau M, Jentsch TJ, Fontaine B (1998) Novel muscle chloride channel (CLCN1) mutations in myotonia congenita with various modes of inheritance including incomplete dominance and penetrance. Neurology 50:1176-1179
93. 93. Ponting CP (1997) CBS domains in ClC chloride channels implicated in myotonia and nephrolithiasis. Mol Med 75:160-163
94. 94. Pusch M (1996) Knocking on channel's door: the permeating chloride ion acts as the gating charge in ClC-0. J Gen Physiol 108:233-236
95. 95. Pusch M, Jentsch TJ (1994) Molecular physiology of voltage-gated chloride channels. Physiol Rev 74:813-827
96. 96. Pusch M, Steinmeyer K, Jentsch TJ (1994) Low single-channel conductance of the major skeletal muscle chloride channel ClC-1. Biophys J 66:149-152
97. 97. Pusch M, Ludewig U, Rehfeldt A, Jentsch TJ (1995) Gating of the voltage-dependent chloride channel ClC-0 by the permeant anion. Nature 373:527-531
98. 98. Pusch M, Steinmeyer K, Koch MC, Jentsch TJ (1995) Mutations in dominant human myotonia congenita drastically alter the voltage-dependence of the ClC-1 chloride channel. Neuron 15:1455-1463
99. 99. Pusch M, Ludewig U, Jentsch TJ (1997). Temperature dependence of fast and slow gating relaxations of ClC-0 chloride channels. J Gen Physiol 109:105-116
100. 100. Pusch M, Jordt SE, Stein V, Jentsch TJ (1999) Chloride dependence of hyperpolarization-activated CLC-gates. J Physiol (Lond) 515:341-353
101. 101. Rychkov GY, Pusch M, Astill ASJ, Roberts ML, Jentsch TJ, Bretag AH (1996) Concentration and pH dependence of skeletal muscle chloride channel ClC-1. J Physiol (Lond) 497.2:423-435
102. 2+ and a carboxylate blocker with the rat ClC-1 chloride channel and its R304E mutant in the Sf-9 insect cell line. J Physiol (Lond) 501:355-362
103. 103. Rychkov Y, Pusch M, Roberts ML, Jentsch TJ, Bretag AH (1998) Permeation and block of the skeletal muscle chloride channel, ClC-1, by foreign anions. J Gen Physiol 111: 653-665
104. 104. Saccomani G, Psarras CG, Smith PR, Kirk KL, Shoemaker RL (1991) Histamine-induced chloride channels in apical membrane of isolated rabbit parietal cells. Am J Physiol 260:C1000-C1011
105. - channels. J Biol Chem 271:10210-10216
106. 106. Saviane C, Conti F, Pusch M (1999) The muscular chloride channel CLC-1 has a double barreled appearance that is differentially affected in dominant and recessive myotonia. J Gen Physiol 113:457-467
107. 107. Schmidt-Rose T, Jentsch TJ (1997) Reconstitution of functional voltage gated chloride channels from complementary fragments of ClC-1. J Biol Chem 272:20515-20521
108. 108. Schmidt-Rose T, Jentsch TJ (1997) Transmembrane topology of a CLC chloride channel. Proc Natl Acad Sci USA 94:7633-7638
109. 109. Schmieder S, Lindenthal S, Banderali U, Ehrenfeld J (1998) Characterization of the putative chloride channel xClC-5 expressed in Xenopus laevis oocytes and comparison with endogenous chloride currents. J Physiol (Lond) 511:379-393
110. 110. Schwappach B, Stobrawa S, Hechenberger M, Steinmeyer K, Jentsch TJ (1998) Golgi localization and functionally important domains at the N-and C-terminus of the yeast CLC putative chloride channel Gef1p. J Biol Chem 273:15110-15118
111. 111. Schwiebert EM, Cid-Soto LP, Stafford T, Carter M, Blaisdell, CJ, Zeitlin PL, Guggino WB, Cutting GR (1998) Analysis of ClC-2 channels as an alternative pathway for chloride conduction in cystic fibrosis airway cells. Proc Natl Acad Sci USA 95:3879-3884
112. 112. Sherry AM, Stroffekova K, Knapp LM, Kupert EY, Cuppoletti J, Malinowska DH (1997) Characterization of the human pH-and PKA-activated ClC-2G(2α) Cl-channel. Am J Physiol 273:C384-C393
113. 113. Simon DB, Bindra RS, Mansfield TA, Nelson-Williams C, Mendonca E, Stone R, Schurman S, Nayir A, Alpay H, Bakkaloglu A, Rodriguez-Soriano J, Morales JM, Sanjad SA, Taylor CM, Pilz D, Brem, A, Trachtman H, Griswold W, Richard GA, John E, Lifton RP (1997) Mutations in the chloride channel gene, CLCNKB, cause Bartter's syndrome type III. Nature Genet 17:171-178
114. 114. Smith RL, Clayton GH, Wilcox CL, Escudero KW, Staley KJ (1995) Differential expression of an inwardly rectifying chloride conductance in rat brain neurons: a potential mechanism for cell-specific modulation of postsynaptic inhibition. J Neurosci 15:4057-4067
115. A receptor function following gene transfer of the ClC-2 chloride channel. Neuron 17:543-551
116. 116. Steinmeyer K, Klocke R, Ortland C, Gronemeier M, Jockusch H, Gründer S, Jentsch TJ (1991) Inactivation of muscle chloride channel by transposon insertion in myotonic mice. Nature 354:304-308
117. 117. Steinmeyer K, Ortland C, Jentsch TJ (1991) Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel. Nature 354:301-304
118. 118. Steinmeyer K, Lorenz C, Pusch M, Koch MC, Jentsch TJ (1994) Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen). EMBO J 13:737-743
119. 119. Steinmeyer K, Schwappach B, Bens M, Vandewalle A, Jentsch TJ (1995) Cloning and functional expression of rat ClC-5, a chloride channel related to kidney disease. J Biol Chem 270:31172-31177
120. 120. Strange K (1998) Molecular identity of the outwardly rectifying swelling-activated anion channel: time to reevaluate pIC-ln. J Gen Physiol 111:617-622
121. 121. Thiemann A, Gründer S, Pusch M, Jentsch, TJ (1992) A chloride channel widely expressed in epithelial and non-epithelial cells. Nature 356:57-60
122. 122. Uchida S, Sasaki S, Furukawa T, Hiraoka M, Imai T, Hirata Y, Marumo F (1993) Molecular cloning of a chloride channel that is regulated by dehydration and expressed predominantly in kidney medulla. J Biol Chem 268:3821-3824 [Erratum: J Biol Chem 269:19192 (1994)]
123. 123. Uchida S, Sasaki S, Nitta K, Uchida K, Horita S, Nihei H, Marumo F (1995) Localization and functional characterization of rat kidney-specific chloride channel, ClC-K1. Am Soc Clin Invest 95:104-134
124. 124. Valverde MA, Diaz M, Sepulveda FV, Gill DR, Hyde SC, Higgins CF (1992) Volume-regulated chloride channels associated with the human multidrug-resistance P-glycoprotein. Nature 355:830-833
125. 125. Vandewalle A, Cluzeaud F, Bens M, Kieferle S, Steinmeyer K, Jentsch TJ (1997) Localization and induction by dehydration of ClC-K chloride channels in the rat kidney. Am J Physiol 272:F678-F688
126. 126. Van Slegtenhorst MA, Bassi MT, Borsani G, Wapenaar MC, Ferrero GB, de Conciliis L, Rugarli EI, Grillo A, Franco B, Zoghni HY, Ballabio A (1994) A gene from the Xp22.3 region shares homology with voltage-gated chloride channels. Hum Mol Genet 3:547-552
127. 127. Wine JJ, Luckie DB (1996) Cell-volume regulation: P-glycoprotein - a cautionary tale. Curr Biol 6:1410-1412
128. 128. Wollnik B, Kubisch C, Steinmeyer K, Pusch M (1997) Identification of functionally important regions of the muscular chloride channel ClC-1 by analysis of recessive and dominant myotonic mutations. Hum Mol Genet 6:805-811
129. - channels. Am J Physiol 270:F1066-F1072