Structure and function of CLC channels

Annual Review of Physiology

Volumn 67, Issue , 2005, Pages 809-839

  Chen, Tsung Yu ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Chloride channel; CLC family; Gating; Permeation; Transporter

Indexed keywords

ANTIPORTER; BACTERIAL PROTEIN; CHLORIDE CHANNEL; CHLORIDE PROTON ANTIPORTER; CLC EC1 PROTEIN; CLC PROTEIN; CYSTEINE; ION CHANNEL; MEMBRANE PROTEIN; UNCLASSIFIED DRUG;

CELL MEMBRANE; CHANNEL GATING; CHLORIDE TRANSPORT; DEPOLARIZATION; ELECTROPHYSIOLOGY; ESCHERICHIA COLI; HYPOTHESIS; ION CONDUCTANCE; ION PERMEABILITY; ION TRANSPORT; MOLECULAR MECHANICS; MULTIGENE FAMILY; PRIORITY JOURNAL; PROTEIN STRUCTURE; REVIEW; SALMONELLA TYPHIMURIUM; STRUCTURE ACTIVITY RELATION; VERTEBRATE;

ANIMALS; BACTERIA; CHLORIDE CHANNELS; HUMANS; ION CHANNEL GATING; STRUCTURE-ACTIVITY RELATIONSHIP; VERTEBRATES;

EID: 15544388091     PISSN: 00664278     EISSN: None     Source Type: Book Series    
DOI: 10.1146/annurev.physiol.67.032003.153012     Document Type: Review

References (166)

1. Koch MC, Steinmeyer K, Lorenz C, Ricker K, Wolf F, et al. 1992. The skeletal muscle chloride channel in dominant and recessive human myotonia. Science 257:797-800
2. Jentsch TJ, Lorenz C, Pusch M, Steinmeyer K. 1995. Myotonias due to CLC-1 chloride channel mutations. Soc. Gen. Physiol. Ser. 50:149-59
3. 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. 7:1753-60
4. Zhang J, Bendahhou S, Sanguinetti MC, Ptacek LJ. 2000. Functional consequences of chloride channel gene (CLCN1) mutations causing myotonia congenita. Neurology 54:937-42
5. George AL Jr, Crackower MA, Abdalla JA, Hudson AJ, Ebers GC. 1993. Molecular basis of Thomsen's disease (autosomal dominant myotonia congenita). Nat. Genet. 3:305-10
6. Lloyd SE, Gunther W, Pearce SH, Thomson A, Bianchi ML, et al. 1997. Characterisation of renal chloride channel, CLCN5, mutations in hypercalciuric nephrolithiasis (kidney stones) disorders. Hum. Mol. Genet. 6:1233-39
7. Lloyd SE, Pearce SH, Fisher SE, Steinmeyer K, Schwappach B, et al. 1996. A common molecular basis for three inherited kidney stone diseases. Nature 379:445-49
8. George AL Jr. 1998. Chloride channels and endocytosis: ClC-5 makes a dent. Proc. Natl. Acad. Sci. USA 95:7843-45
9. Konrad M, Vollmer M, Lemmink HH, van den Heuvel LP, Jeck N, et al. 2000. Mutations in the chloride channel gene CLC-NKB as a cause of classic Bartter syndrome. J. Am. Soc. Nephrol. 11:1449-59
10. Thakker RV. 2000. Molecular pathology of renal chloride channels in Dent's disease and Bartter's syndrome. Exp. Nephrol. 8:351-60
11. Hebert SC. 2003. Bartter syndrome. Curr. Opin. Nephrol. Hypertens. 12:527-32
12. Kornak U, Kasper D, Bosl MR, Kaiser E, Schweizer M, et al. 2001. Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man. Cell 104:205-15
13. Haug K, Warnstedt M, Alekov AK, Sander T, Ramirez A, et al. 2003. Mutations in CLCN2 encoding a voltage-gated chloride channel are associated with idiopathic generalized epilepsies. Nat. Genet. 33:527-32
14. Stobrawa SM, Breiderhoff T, Takamori S, Engel D, Schweizer M, et al. 2001. Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus. Neuron 29:185-96
15. - channel disruption. EMBO J. 20:1289-99
16. Jentsch TJ, Friedrich T, Schriever A, Yamada H. 1999. The CLC chloride channel family. Pflügers Arch. 437:783-95
17. Jentsch TJ, Stein V, Weinreich F, Zdebik AA. 2002. Molecular structure and physiological function of chloride channels. Physiol. Rev. 82:503-68
18. Maduke M, Miller C, Mindell JA. 2000. A decade of CLC chloride channels: structure, mechanism, and many unsettled questions. Annu. Rev. Biophys. Biomol. Struct. 29:411-38
19. Waldegger S, Jentsch TJ. 2000. From tonus to tonicity: physiology of CLC chloride channels. J. Am. Soc. Nephrol. 11:1331-39
20. - channels gleaned from human genetic disease and mouse models. Annu. Rev. Physiol. 67:779-807
21. Jentsch TJ, Steinmeyer K, Schwarz G. 1990. Primary structure of Torpedo marmorata chloride channel isolated by expression cloning in Xenopus oocytes. Nature 348:510-14
22. Purdy MD, Wiener MC. 2000. Expression, purification, and initial structural characterization of YadQ, a bacterial homolog of mammalian ClC chloride channel proteins. FEBS Lett. 466:26-28
23. Maduke M, Pheasant DJ, Miller C. 1999. High-level expression, functional reconstitution, and quaternary structure of a prokaryotic ClC-type chloride channel. J. Gen. Physiol. 114:713-22
24. Iyer R, Iverson TM, Accardi A, Miller C. 2002. A biological role for prokaryotic ClC chloride channels. Nature 419:715-18
25. Schwappach B, Stobrawa S, Hechenberger M, Steinmeyer K, Jentsch TJ, 1998. Golgi localization and functionally important domains in the NH2 and COOH terminus of the yeast CLC putative chloride channel Gef1p. J. Biol. Chem. 273:15110-18
26. Gaxiola RA, Yuan DS, Klausner RD, Fink GR. 1998. The yeast CLC chloride channel functions in cation homeostasis. Proc. Natl. Acad. Sci. USA 95:4046-50
27. Hechenberger M, Schwappach B, Fischer WN, Frommer WB, Jentsch TJ, Steinmeyer K. 1996. A family of putative chloride channels from Arabidopsis and functional complementation of a yeast strain with a CLC gene disruption. J. Biol. Chem. 271:33632-38
28. Schriever AM, Friedrich T, Pusch M, Jentsch TJ. 1999. CLC chloride channels in Caenorhabditis elegans. J. Biol. Chem. 274:34238-44
29. Strange K. 2002. Of mice and worms: novel insights into ClC-2 anion channel physiology. News Physiol. Sci. 17:11-16
30. Bianchi L, Miller DM 3rd, George AL Jr. 2001. Expression of a CIC chloride channel in Caenorhabditis elegans gamma-aminobutyric acid-ergic neurons. Neurosci. Lett. 299:177-80
31. Nehrke K, Begenisich T, Pilato J, Melvin JE. 2000. Into ion channel and transporter function. Caenorhabditis elegans ClC-type chloride channels: novel variants and functional expression. Am. J. Physiol. Cell Physiol. 279:C2052-66
32. Gunther W, Piwon N, Jentsch TJ. 2003. The ClC-5 chloride channel knock-out mouse-an animal model for Dent's disease. Pflügers Arch. 445:456-62
33. Mohammad-Panah R, Harrison R, Dhani S, Ackerley C, Huan LJ, et al. 2003. The chloride channel ClC-4 contributes to endosomal acidification and trafficking. J. Biol. Chem. 278:29267-77
34. Duan D, Winter C, Cowley S, Hume JR, Horowitz B. 1997. Molecular identification of a volume-regulated chloride channel. Nature 390:417-21
35. Duan D, Zhong J, Hermoso M, Satterwhite CM, Rossow CF, et al. 2001. Functional inhibition of native volume-sensitive outwardly rectifying anion channels in muscle cells and Xenopus oocytes by anti-ClC-3 antibody. J. Physiol. 531:437-44
36. -/- mice. J. Physiol. 557:439-56
37. - channels and ClC-3 in acinar cells isolated from the rat lacrimal gland and submandibular salivary gland. J. Physiol. 534:409-21
38. Weylandt KH, Valverde MA, Nobles M, Raguz S, Amey JS, et al. 2001. Human ClC-3 is not the swelling-activated chloride channel involved in cell volume regulation. J. Biol. Chem. 276:17461-67
39. Brandt S, Jentsch TJ. 1995. ClC-6 and ClC-7 are two novel broadly expressed members of the CLC chloride channel family. FEBS Lett. 377:15-20
40. - channels. J. Gen. Physiol 123:109-19
41. - channels. Nature 427:803-7
42. Mindell JA, Maduke M, Miller C, Grigorieff N. 2001. Projection structure of a ClC-type chloride channel at 6.5 Å resolution. Nature 409:219-23
43. Dutzler R, Campbell EB, Cadene M, Chait BT, MacKinnon R. 2002. X-ray structure of a ClC chloride channel at 3.0 Å reveals the molecular basis of anion selectivity. Nature 415:287-94
44. Dutzler R, Campbell EB, MacKinnon R. 2003. Gating the selectivity filter in ClC chloride channels. Science 300:108-12
45. O'Neill GP, Grygorczyk R, Adam M, Ford-Hutchinson AW. 1991. The nucleotide sequence of a voltage-gated chloride channel from the electric organ of Torpedo californica. Biochim. Biophys. Acta 1129:131-34
46. Steinmeyer K, Ortland C, Jentsch TJ. 1991. Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel. Nature 354:301-4
47. Thiemann A, Grunder S, Pusch M, Jentsch TJ. 1992. A chloride channel widely expressed in epithelial and non-epithelial cells. Nature 356:57-60
48. 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 88:11052-56
49. Lin CW, Chen TY. 2000. Cysteine modification of a putative pore residue in ClC-0: implication for the pore stoichiometry of ClC chloride channels. J. Gen. Physiol. 116:535-46
50. Ludewig U, Pusch M, Jentsch TJ. 1996. Two physically distinct pores in the dimeric ClC-0 chloride channel. Nature 383:340-43
51. Ludewig U, Pusch M, Jentsch TJ. 1997. Independent gating of single pores in CLC-0 chloride channels. Biophys. J. 73:789-97
52. Middleton RE, Pheasant DJ, Miller C. 1994. Purification, reconstitution, and subunit composition of a voltage-gated chloride channel from Torpedo electroplax. Biochemistry 33:13189-98
53. Middleton RE, Pheasant DJ, Miller C. 1996. Homodimeric architecture of a ClC-type chloride ion channel. Nature 383:337-40
54. Miller C. 1982. Open-state substructure of single chloride channels from Torpedo electroplax. Philos. Trans. R. Soc. London Ser B 299:401-11
55. Miller C, White MM. 1984. Dimeric structure of single chloride channels from Torpedo electroplax. Proc. Natl. Acad. Sci. USA 81:2772-75
56. Weinreich F, Jentsch TJ. 2001. Pores formed by single subunits in mixed dimers of different CLC chloride channels. J. Biol. Chem. 276:2347-53
57. Faraldo-Gomez JD, Roux B. 2004. Electrostatics of ion stabilization in a ClC chloride channel homologue from Escherichia coli. J. Mol. Biol 339:981-1000
58. Lin CW, Chen TY. 2003. Probing the pore of ClC-0 by substituted cysteine accessibility method using methane thiosulfonate reagents. J. Gen. Physiol. 122:147-59
59. Estevez R, Schroeder BC, Accardi A, Jentsch TJ, Pusch M. 2003. Conservation of chloride channel structure revealed by an inhibitor binding site in ClC-1. Neuron 38:47-59
60. Miller C. 2003. ClC channels: reading eukaryotic function through prokaryotic spectacles. J. Gen. Physiol. 122:129-31
61. + secretion. Nature 414:558-61
62. Waldegger S, Jeck N, Earth P, Peters M, Vitzthum H, et al. 2002. Barttin increases surface expression and changes current properties of ClC-K channels. Pflügers Arch. 444:411-18
63. Vanoye CG, George AL Jr. 2002. Functional characterization of recombinant human ClC-4 chloride channels in cultured mammalian cells. J. Physiol 539:373-83
64. 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
65. Uchida S, Sasaki S. 2005. Function of chloride channels in the kidney. Annu. Rev. Physiol. 67:759-78
66. Hanke W, Miller C. 1983. Single chloride channels from Torpedo electroplax. Activation by protons. J. Gen. Physiol. 82:25-45
67. Saviane C, Conti F, Pusch M. 1999. The muscle chloride channel ClC-1 has a double-barreled appearance that is differentially affected in dominant and recessive myotonia. J. Gen. Physiol. 113:457-68
68. Miller C, White MM. 1980. A voltage-dependent chloride conductance channel from Torpedo electroplax membrane. Ann. NY Acad. Sci. 341:534-51
69. White MM, Miller C. 1981. Chloride permeability of membrane vesicles isolated from Torpedo californica electroplax. Biophys. J. 35:455-62
70. Richard EA, Miller C. 1990. Steady-state coupling of ion-channel conformations to a transmembrane ion gradient. Science 247:1208-10
71. - channel. J. Gen. Physiol. 108:237-50
72. Pusch M, Ludewig U, Jentsch TJ. 1997. Temperature dependence of fast and slow gating relaxations of ClC-0 chloride channels. J. Gen. Physiol. 109:105-16
73. Chen TY. 1998. Extracellular zinc ion inhibits ClC-0 chloride channels by facilitating slow gating. J. Gen. Physiol. 112:715-26
74. Lin YW, Lin CW, Chen TY. 1999. Elimination of the slow gating of ClC-0 chloride channel by a point mutation. J. Gen. Physiol. 114:1-12
75. 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. Cell Physiol. 274:C966-73
76. Hryciw DH, 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. 273:4304-7
77. Hebeisen S, Biela A, Giese B, Muller-Newen G, Hidalgo P, Fahlke C. 2004. The role of the carboxy-terminus in ClC chloride channel function. J. Biol. Chem. 279:13140-47
78. Estevez R, Pusch M, Ferrer-Costa C, Orozco M, Jentsch TJ. 2004. Functional and structural conservation of CBS domains from CLC channels. J. Physiol. 557:363-78
79. Maduke M, Williams C, Miller C. 1998. Formation of CLC-0 chloride channels from separated transmembrane and cytoplasmic domains. Biochemistry 37:1315-21
80. Schmidt-Rose T, Jentsch TJ. 1997. Reconstitution of functional voltage-gated chloride channels from complementary fragments of CLC-1. J. Biol. Chem. 272:20515-21
81. Accardi A, Pusch M. 2000. Fast and slow gating relaxations in the muscle chloride channel CLC-1. J. Gen. Physiol. 116:433-44
82. Aromataris EC, Rychkov GY, Bennetts B, Hughes BP, Bretag AH, Roberts ML. 2001. Fast and slow gating of CLC-1: differential effects of 2-(4-chlorophenoxy) propionic acid and dominant negative mutations. Mol. Pharmacol. 60:200-8
83. Bennetts B, Roberts ML, Bretag AH, Rychkov GY. 2001. Temperature dependence of human muscle ClC-1 chloride channel. J. Physiol. 535:83-93
84. Accardi A, Ferrera L, Pusch M. 2001. Drastic reduction of the slow gate of human muscle chloride channel (ClC-1) by mutation C277S. J. Physiol. 534:745-52
85. 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. 501:355-62
86. 2+ blockade of the human skeletal muscle chloride channel. J. Biol. Chem. 274:11687-92
87. 2+ and other sulfhydryl-reactive compounds. Pflügers Arch. 433:357-63
88. Duffield M, Rychkov G, Bretag A, Roberts M. 2003. Involvement of helices at the dimer interface in ClC-1 common gating. J. Gen. Physiol. 121:149-61
89. Grunder S, Thiemann A, Pusch M, Jentsch TJ. 1992. Regions involved in the opening of ClC-2 chloride channel by voltage and cell volume. Nature 360:759-62
90. Jordt SE, Jentsch TJ. 1997. Molecular dissection of gating in the ClC-2 chloride channel. EMBO J. 16:1582-92
91. Hoshi T, Zagotta WN, Aldrich RW. 1990. Biophysical and molecular mechanisms of Shaker potassium channel inactivation. Science 250:533-38
92. Zagotta WN, Hoshi T, Aldrich RW. 1990. Restoration of inactivation in mutants of Shaker potassium channels by a peptide derived from ShB. Science 250:568-71
93. + channels. Proc. Natl. Acad. Sci. USA 88:5092-95
94. + channel behaves like an open-channel blocker. Neuron 7:743-53
95. Varela D, Niemeyer MI, Cid LP, Sepulveda FV. 2002. Effect of an N-terminus deletion on voltage-dependent gating of the ClC-2 chloride channel. J. Physiol. 544:363-72
96. Zuniga L, Niemeyer MI, Varela D, Catalan M, Cid LP, Sepulveda FV. 2004. The voltage-dependent ClC-2 chloride channel has a dual gating mechanism. J. Physiol. 555:671-82
97. Pusch M, Ludewig U, Rehfeldt A, Jentsch TJ. 1995. Gating of the voltage-dependent chloride channel CIC-0 by the permeant anion. Nature 373:527-31
98. Rychkov GY, Pusch M, Astill DS, Roberts ML, Jentsch TJ, Bretag AH. 1996. Concentration and pH dependence of skeletal muscle chloride channel ClC-1. J. Physiol. 497:423-35
99. + of the muscle-type ClC chloride channels. J. Gen. Physiol. 118:23-32
100. + channel. Nature 423:42-48
101. + channel. Nature 423:33-41
102. Fahlke C, Rosenbohm A, Mitrovic N, George AL Jr, Rudel R. 1996. Mechanism of voltage-dependent gating in skeletal muscle chloride channels. Biophys. J. 71:695-706
103. Fahlke C, Rudel R, Mitrovic N, Zhou M, George AL Jr. 1995. An aspartic acid residue important for voltage-dependent gating of human muscle chloride channels. Neuron 15:463-72
104. Ludewig U, Jentsch TJ, Pusch M. 1997. Inward rectification in ClC-0 chloride channels caused by mutations in several protein regions. J. Gen. Physiol. 110:165-71
105. Rychkov GY, 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. 111:653-65
106. Pusch M. 2004. Structural insights into chloride and proton-mediated gating of CLC chloride channels. Biochemistry 43:1135-44
107. Pusch M, Jordt SE, Stein V, Jentsch TJ. 1999. Chloride dependence of hyperpolarization-activated chloride channel gates. J. Physiol. 515:341-53
108. Niemeyer MI, Cid LP, Zuniga L, Catalan M, Sepulveda FV. 2003. A conserved pore-lining glutamate as a voltage-and chloride-dependent gate in the ClC-2 chloride channel. J. Physiol. 553:873-79
109. Fahlke C, Rhodes TH, Desai RR, George AL Jr. 1998. Pore stoichiometry of a voltage-gated chloride channel. Nature 394:687-90
110. Warnstedt M, Sun C, Poser B, Escriva MJ, Tranebjaerg L, et al. 2002. The myotonia congenita mutation A331T confers a novel hyperpolarization-activated gate to the muscle chloride channel ClC-1. J. Neurosci. 22:7462-70
111. Zhang J, Sanguinetti MC, Kwiecinski H, Ptacek LJ. 2000. Mechanism of inverted activation of ClC-1 channels caused by a novel myotonia congenita mutation. J. Biol. Chem. 275:2999-3005
112. Chen TY, Chen MF, Lin CW. 2003. Electrostatic control and chloride regulation of the fast gating of ClC-0 chloride channels. J. Gen. Physiol. 122:641-51
113. +. Nature 291:427-29
114. Fahlke C, Durr C, George AL Jr. 1997. Mechanism of ion permeation in skeletal muscle chloride channels. J. Gen. Physiol. 110:551-64
115. Fahlke C, Yu HT, Beck CL, Rhodes TH, George AL Jr. 1997. Pore-forming segments in voltage-gated chloride channels. Nature 390:529-32
116. 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. 498:691-702
117. Hebeisen S, Heidtmann H, Cosmelli D, Gonzalez C, Poser B, et al. 2003. Anion permeation in human ClC-4 channels. Biophys. J. 84:2306-18
118. + channel. J. Gen. Physiol. 92:569-86
119. Neyton J, Miller C. 1988. Potassium blocks barium permeation through a calcium-activated potassium channel. J. Gen. Physiol. 92:549-67
120. 2+ in rat phaeochromocytoma cells. J. Physiol. 466:683-706
121. 2+ channel pore in rat phaeochromocytoma cells. J. Physiol. 466:657-82
122. 2+ channel in rat phaeochromocytoma cells: two sets of ion binding sites in the pore. J. Physiol. 466:629-55
123. Pusch M, Steinmeyer K, Jentsch TJ. 1994. Low single channel conductance of the major skeletal muscle chloride channel, ClC-1. Biophys. J. 66:149-52
124. - pores: electrostatic effects of charged residues. Biophys. J. 86:825-35
125. Cohen J, Schulten K. 2004. Mechanism of anionic conduction across ClC. Biophys. J. 86:836-45
126. Corry B, O'Mara M, Chung SH. 2004. Conduction mechanisms of chloride ions in ClC-type channels. Biophys. J. 86:846-60
127. 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. 13:737-43
128. Pusch M, Steinmeyer K, Koch MC, Jentsch TJ. 1995. Mutations in dominant human myotonia congenita drastically alter the voltage dependence of the CIC-1 chloride channel. Neuron 15:1455-63
129. Waldegger S, Jentsch TJ. 2000. Functional and structural analysis of ClC-K chloride channels involved in renal disease. J. Biol Chem. 275:24527-33
130. Chen TY. 2003. Coupling gating with ion permeation in ClC channels. SciSTKE 2003:pe23
131. Akabas MH, Stauffer DA, Xu M, Karlin A. 1992. Acetylcholine receptor channel structure probed in cysteine-substitution mutants. Science 258:307-10
132. + channel. Neuron 19:175-84
133. Yang N, George AL Jr, Horn R. 1996. Molecular basis of charge movement in voltage-gated sodium channels. Neuron 16:113-22
134. + channel S4. Neuron 16:387-97
135. Wilson GG, Pascual JM, Brooijmans N, Murray D, Karlin A. 2000. The intrinsic electrostatic potential and the intermediate ring of charge in the acetylcholine receptor channel. J. Gen. Physiol. 115:93-106
136. Pascual JM, Karlin A. 1998. State-dependent accessibility and electrostatic potential in the channel of the acetylcholine receptor. Inferences from rates of reaction of thiosulfonates with substituted cysteines in the M2 segment of the alpha subunit. J. Gen. Physiol. 111:717-39
137. Fahlke C, Desai RR, Gillani N, George AL Jr. 2001. Residues lining the inner pore vestibule of human muscle chloride channels. J. Biol. Chem. 276:1759-65
138. Chen MF, Chen TY. 2003. Side-chain charge effects and conductance determinants in the pore of ClC-0 chloride channels. J. Gen. Physiol. 122:133-45
139. + channel. Biochemistry 28:8092-99
140. + channel by trimethyloxonium. Biochemistry 28:8087-92
141. MacKinnon R, Miller C. 1989. Mutant potassium channels with altered binding of charybdotoxin, apore-blockingpeptide inhibitor. Science 245:1382-85
142. + channel. J. Gen. Physiol. 91:335-49
143. + channel. Science 251:939-42
144. + channels. Science 250:276-79
145. Pusch M, Accardi A, Liantonio A, Guida P, Traverse S, et al. 2002. Mechanisms of block of muscle type CLC chloride channels. Mol. Membr. Biol. 19:285-92
146. Pusch M, Accardi A, Liantonio A, Ferrera L, De Luca A, et al. 2001. Mechanism of block of single protopores of the Torpedo chloride channel ClC-0 by 2-(p-chlorophenoxy)butyric acid (CPB). J. Gen. Physiol. 118:45-62
147. DeLuca A, Tricarico D, Wagner R, Bryant SH, Tortorella V, Conte Camerino D. 1992. Opposite effects of enantiomers of clofibric acid derivative on rat skeletal muscle chloride conductance: antagonism studies and theoretical modeling of two different receptor site interactions. J. Pharmacol. Exp. Ther. 260:364-68
148. Pusch M, Liantonio A, Bertorello L, Accardi A, De Luca A, et al. 2000. Pharmacological characterization of chloride channels belonging to the ClC family by the use of chiral clofibric acid derivatives. Mol. Pharmacol. 58:498-507
149. Liantonio A, De Luca A, Pierno S, Didonna MP, Loiodice F, et al. 2003. Structural requisites of 2-(p-chlorophenoxy) propionic acid analogues for activity on native rat skeletal muscle chloride conductance and on heterologously expressed CLC-1. Br. J. Pharmacol. 139:1255-64
150. Liantonio A, Accardi A, Carbonara G, Fracchiolla G, Loiodice F, et al. 2002. Molecular requisites for drug binding to muscle CLC-1 and renal CLC-K channel revealed by the use of phenoxy-alkyl derivatives of 2-(p-chlorophenoxy) propionic acid. Mol. Pharmacol. 62:265-71
151. Aromataris EC, Astill DS, Rychkov GY, Bryant SH, Bretag AH, Roberts ML. 1999. Modulation of the gating of ClC-1 by S-(-) 2-(4-chlorophenoxy) propionic acid. Br. J. Pharmacol. 126:1375-82
152. Accardi A, Pusch M. 2003. Conformational changes in the pore of CLC-0. J. Gen. Physiol. 122:277-93
153. Traverso S, Elia L, Pusch M. 2003. Gating competence of constitutively open CLC-0 mutants revealed by the interaction with a small organic inhibitor. J. Gen. Physiol. 122:295-306
154. Harris GL, Betz WJ. 1987. Evidence for active chloride accumulation in normal and denervated rat lumbrical muscle. J. Gen. Physiol. 90:127-44
155. Betz WJ, Caldwell JH, Harris GL. 1986. Effect of denervation on a steady electric current generated at the end-plate region of rat skeletal muscle. J. Physiol. 373:97-114
156. Betz WJ, Caldwell JH, Kinnamon SC. 1984. Physiological basis of a steady endogenous current in rat lumbrical muscle. J. Gen. Physiol. 83:175-92
157. Li X, Wang T, Zhao Z, Weinman SA. 2002. The ClC-3 chloride channel promotes acidification of lysosomes in CHO-K1 and Huh-7 cells. Am. J. Physiol. Cell Physiol. 282:C1483-91
158. Zhou Z, Hu S, Hwang TC. 2002. Probing an open CFTR pore with organic anion blockers. J. Gen. Physiol. 120:647-62
159. Zou XQ, Hwang TC. 2001. ATP hydrolysis-coupled gating of CFTR chloride channels: structure and function. Biochemistry 40:5579-86
160. +-pump ligands modulate gating of paly-toxin-induced ion channels. Proc. Natl. Acad. Sci. USA 100:501-5
161. + pump. Ann. NY Acad. Sci. 976:31-40
162. Ryan RM, Vandenberg RJ. 2002. Distinct conformational states mediate the transport and anion channel properties of the glutamate transporter EAAT-1. J. Biol. Chem. 277:13494-500
163. Slotboom DJ, Konings WN, Lolkema JS. 2001. Glutamate transporters combine transporter- and channel-like features. Trends Biochem. Sci. 26:534-39
164. Fairman WA, Vandenberg RJ, Arriza JL, Kavanaugh MP, Amara SG. 1995. An excitatory amino-acid transporter with properties of a ligand-gated chloride channel. Nature 375:599-603
165. Wadiche JI, Amara SG, Kavanaugh MP. 1995. Ion fluxes associated with excitatory amino acid transport. Neuron 15:721-28
166. Eskandari S, Kreman M, Kavanaugh MP, Wright EM, Zampighi GA. 2000. Pentameric assembly of a neuronal glutamate transporter. Proc. Natl. Acad. Sci. USA 97:8641-46