CLC chloride channels and transporters: A biophysical and physiological perspective

Reviews of Physiology, Biochemistry and Pharmacology

Volumn 158, Issue , 2007, Pages 23-76

  Zifarelli, G ^a   Pusch, M ^a  

^a CNR   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

4 AMINOBUTYRIC ACID; CALCIUM; CARRIER PROTEIN; CHLORIDE CHANNEL;

ANIMAL; CHANNEL GATING; CHEMICAL STRUCTURE; CHEMISTRY; HUMAN; METABOLISM; PHYSIOLOGY; PROTEIN CONFORMATION; REVIEW; TRANSPORT AT THE CELLULAR LEVEL;

ANIMALS; BIOLOGICAL TRANSPORT; CALCIUM; CHLORIDE CHANNELS; GAMMA-AMINOBUTYRIC ACID; HUMANS; ION CHANNEL GATING; MEMBRANE TRANSPORT PROTEINS; MODELS, MOLECULAR; PROTEIN CONFORMATION;

EID: 34548852606     PISSN: 03034240     EISSN: None     Source Type: Book Series    
DOI: 10.1007/112_2006_0605     Document Type: Article

References (294)

1. Abramson J, Smirnova I, Kasho V, Verner G, Kaback HR, Iwata S (2003) Structure and mechanism of the lactose permease of Escherichia coli. Science 301:610-615
2. + channel by Nedd4 in Liddle's syndrome. J Clin Invest 103:667-673
3. 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 534:745-752
4. - channels. Nature 427:803-807
5. Accardi A, Pusch M (2000) Fast and slow gating relaxations in the muscle chloride channel CLC-1. J Gen Physiol 116:433-444
6. Accardi A, Pusch M (2003) Conformational changes in the pore of CLC-0. J Gen Physiol 122:277-293
7. + exchanger. J Gen Physiol 126:563-570.
8. Adrian RH, Bryant SH (1974) On the repetitive discharge in myotonic muscle fibres. J Physiol 240:505-515
9. +-induced anomalous rectification in squid giant axons. J Gen Physiol 50:491-503
10. 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 126:1375-1382
11. 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 60:200-208
12. Arreola J, Begenisich T, Melvin JE (2002) Conformation-dependent regulation of inward rectifier chloride channel gating by extracellular protons. J Physiol 541:103-112
13. Arreola J, Melvin JE (2003) A novel chloride conductance activated by extracellular ATP in mouse parotid acinar cells. J Physiol 547:197-208
14. Bali M, Lipecka J, Edelman A, Fritsch J (2001) Regulation of ClC-2 chloride channels in T84 cells by TGF-α. Am J Physiol Cell Physiol 280:C1588-C1598
15. Barbier-Brygoo H, Vinauger M, Colcombet J, Ephritikhine G, Frachisse J, Maurel C (2000) Anion channels in higher plants:fUnctional characterization, molecular structure and physiological role. Biochim Biophys Acta 1465:199-218
16. Bateman A (1997) The structure of a domain common to archaebacteria and the homocystinuria disease protein. Trends Biochem Sci 22:12-13
17. 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
18. Becker PE (1957) Zur Frage der Heterogenie der erblichen Myotonien. Nervenarzt 28:455-460
19. Bennekou P, de Franceschi L, Pedersen O, Lian L, Asakura T, Evans G, Brugnara C, Christophersen P (2001) Treatment with NS3623, a novel Cl- conductance blocker, ameliorates erythrocyte dehydration in transgenic SAD mice:a possible new therapeutic approach for sickle cell disease. Blood 97:1451-1457
20. Bennetts B, Roberts ML, Bretag AH, Rychkov GY (2001) Temperature dependence of human muscle ClC-1 chloride channel. J Physiol 535:83-93
21. Bennetts B, Rychkov GY, Ng H-L, Morton CJ, Stapleton D, Parker MW, Cromer BA (2005) Cytoplasmic ATP-sensing domains regulate gating of skeletal muscle ClC-1 chloride channels. J Biol Chem 280:32452-32458
22. Berg J, Jiang H, Thornton CA, Cannon SC (2004) Truncated ClC-1 mRNA in myotonic dystrophy exerts a dominant-negative effect on the Cl current. Neurology 63:2371-2375
23. Birkenhäger R, Otto E, Schurmann MJ, Vollmer M, Ruf EM,Maier-Lutz I, Beekmann F, Fekete A, Omran H, Feldmann D, Milford DV, Jeck N, Konrad M, Landau D, Knoers NV, Antignac C, Sudbrak R, Kispert A, Hildebrandt F (2001) Mutation of BSND causes Bartter syndrome with sensorineural deafness and kidney failure. Nat Genet 29:310-314
24. Bisset D, Corry B, Chung SH (2005) The fast gating mechanism in ClC-0 channels. Biophys J 89:179-186
25. Blaisdell CJ, Edmonds RD, Wang XT, Guggino S, Zeitlin PL (2000) pH-regulated chloride secretion in fetal lung epithelia. Am J Physiol Lung Cell Mol Physiol 278:L1248-L1255
26. - channel disruption. EMBO J 20:1289-1299
27. 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
28. Bretag AH (1987) Muscle chloride channels. Physiol Rev 67:618-724
29. Cannon SC (2000) Spectrum of sodium channel disturbances in the nondystrophic myotonias and periodic paralyses. Kidney Int 57:772-779
30. Carr G, Simmons NL, Sayer JA (2006) Disruption of clc-5 leads to a redistribution of annexin A2 and promotes calcium crystal agglomeration in collecting duct epithelial cells. Cell Mol Life Sci 63:367-377
31. Catalán M, Cornejo I, Figueroa CD, Niemeyer MI, Sepúlveda FV, Cid LP (2002) ClC-2 in guinea pig colon:mRNA, immunolabeling, and functional evidence for surface epithelium localization. Am J Physiol Gastrointest Liver Physiol 283:G1004-G1013
32. Catalán M, Niemeyer MI, Cid LP, Sepúlveda FV (2004) Basolateral ClC-2 chloride channels in surface colon epithelium:rEgulation by a direct effect of intracellular chloride. Gastroenterology 126:1104-1114
33. Charlet BN, Savkur RS, Singh G, Philips AV, Grice EA, Cooper TA (2002) Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy due to misregulated alternative splicing. Mol Cell 10:45-53
34. + of the muscle- type ClC chloride channels. J Gen Physiol 118:23-32
35. Chen MF, Chen TY (2003) Side-chain charge effects and conductance determinants in the pore of ClC-0 chloride channels. J Gen Physiol 122:133-145
36. - channel 1 gene dosage. J Physiol 504:75-81
37. Chen TY (1998) Extracellular zinc ion inhibits ClC-0 chloride channels by facilitating slow gating. J Gen Physiol 112:715-726
38. Chen TY (2003) Coupling gating with ion permeation in ClC channels. Sci STKE 2003:pE23
39. Chen TY, Chen MF, Lin CW (2003) Electrostatic control and chloride regulation of the fast gating of ClC-0 chloride channels. J Gen Physiol 122:641-651
40. Chen TY, Miller C (1996) Nonequilibrium gating and voltage dependence of the ClC-0 Cl- channel. J Gen Physiol 108:237-250
41. Christensen EI, Devuyst O, Dom G, Nielsen R, Van der Smissen P, Verroust P, Leruth M, Guggino WB, Courtoy PJ (2003) Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules. Proc Natl Acad Sci USA 100:8472-8477
42. Chu S, Blaisdell CJ, Liu M-ZM, Zeitlin PL (1999) Perinatal regulation of the ClC-2 chloride channel in lung is mediated by Sp1 and Sp3. Am J Physiol Lung Cell Mol Physiol 276:L614-L624
43. Clark S, Jordt SE, Jentsch TJ, Mathie A (1998) Characterization of the hyperpolarization-activated chloride current in dissociated rat sympathetic neurons. J Physiol 506:665-678
44. Cleiren E, Benichou O, Van Hul E, Gram J, Bollerslev J, Singer FR, Beaverson K, Aledo A, Whyte MP, Yoneyama T, deVernejoul MC, Van Hul W (2001) Albers-Schonberg disease (autosomal dominant osteopetrosis, type II) results from mutations in the ClCN7 chloride channel gene. Hum Mol Genet 10:2861-2867
45. Cohen J, Schulten K (2004) Mechanism of anionic conduction across ClC. Biophys J 86:836-845
46. Conte-Camerino D, Mambrini M, DeLuca A, Tricarico D, Bryant SH, Tortorella V, Bettoni G (1988) Enantiomers of clofibric acid analogs have opposite actions on rat skeletal muscle chloride channels. Pflügers Arch 413:105-107
47. Corry B, O'Mara M, Chung SH (2004) Conduction mechanisms of chloride ions in ClC-type channels. Biophys J 86:846-860
48. Cupers P, Veithen A, Hoekstra D, Baudhuin P, Courtoy PJ (1997) Three unrelated perturbations similarly uncouple fluid, bulk-membrane, and receptor endosomal flow in rat fetal fibroblasts. Biochem Biophys Res Commun 236:661-664
49. De Angeli A, Monachello D, Ephritikhine G, Frachisse JM, Thomine S, Gambale F, Barbier-Brygoo H (2006) AtCLCa, a proton/nitrate antiporter, mediates nitrate accumulation in plant vacuoles. Nature. In press.
50. de Santiago JA, Nehrke K, Arreola J (2005) Quantitative analysis of the voltage-dependent gating of mouse parotid ClC-2 chloride channel. J Gen Physiol 126:591-603
51. Dent CE, Friedman M (1964) Hypercalcuric rickets associated with renal tubular damage. Arch Dis Child 39:240-249
52. 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
53. Devuyst O, Jouret F, Auzanneau C, Courtoy PJ (2005) Chloride channels and endocytosis:nEw insights from Dent's disease and ClC-5 knockout mice. Nephron Physiol 99:69-73
54. Dhani SU, Bear CE (2006) Role of intramolecular and intermolecular interactions in ClC channel and transporter function. Pflg̈ers Arch 451:708-715
55. Dhani SU, Mohammad-Panah R, Ahmed N, Ackerley C, Ramjeesingh M, Bear CE (2003) Evidence for a functional interaction between the ClC-2 chloride channel and the retrograde motor dynein complex. J Biol Chem 278:16262-16270
56. + conduction and selectivity. Science 280:69-77
57. Duan D, Winter C, Cowley S, Hume JR, Horowitz B (1997) Molecular identification of a volume-regulated chloride channel. Nature 390:417-421
58. Duffield M, Rychkov G, Bretag A, Roberts M (2003) Involvement of helices at the dimer interface in ClC-1 common gating. J Gen Physiol 121:149-161
59. Duffield MD, Rychkov GY, Bretag AH, Roberts ML (2005) Zinc inhibits human ClC-1 muscle chloride channel by interacting with its common gating mechanism. J Physiol 568:5-12
60. Dutzler R (2004) The structural basis of ClC chloride channel function. Trends Neurosci 27:315-320
61. 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 415:287-294
62. Dutzler R, Campbell EB, MacKinnon R (2003) Gating the selectivity filter in ClC chloride channels. Science 300:108-112
63. Edmonds RD, Silva IV, Guggino WB, Butler RB, Zeitlin PL, Blaisdell CJ (2002) ClC-5:oNtogeny of an alternative chloride channel in respiratory epithelia. Am J Physiol Lung Cell Mol Physiol 282:L501-L507
64. Eggermont J, Trouet D, Carton I, Nilius B (2001) Cellular function and control of volume-regulated anion channels. Cell Biochem Biophys 35:263-274
65. Embark HM, Bohmer C, Palmada M, Rajamanickam J,Wyatt AW, Wallisch S, Capasso G, Waldegger P, Seyberth HW, Waldegger S, Lang F (2004) Regulation of CLC-Ka/barttin by the ubiquitin ligase Nedd4-2 and the serum- and glucocorticoid-dependent kinases. Kidney Int 66:1918-1925
66. Engh AM, Maduke M (2005) Cysteine accessibility in ClC-0 supports conservation of the ClC intracellular vestibule. J Gen Physiol 125:601-617
67. + secretion. Nature 414:558-561
68. Estévez R, Jentsch TJ (2002) CLC chloride channels:cOrrelating structure with function. Curr Opin Struct Biol 12:531-539
69. Estévez R, Pusch M, Ferrer-Costa C, Orozco M, Jentsch TJ (2004) Functional and structural conservation of CBS domains from CLC channels. J Physiol 557:363-378
70. Estévez 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
71. 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
72. 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
73. Fölsch H, Ohno H, Bonifacino JS, Mellman I (1999) A novel clathrin adaptor complex mediates basolateral targeting in polarized epithelial cells. Cell 99:189-198
74. 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-C973
75. Frattini A, Pangrazio A, Susani L, Sobacchi C, Mirolo M, Abinun M, Andolina M, Flanagan A, Horwitz EM, Mihci E, Notarangelo LD, Ramenghi U, Teti A, Van Hove J, Vujic D, Young T, Albertini A, Orchard PJ, Vezzoni P, Villa A (2003) Chloride channel ClCN7 mutations are responsible for severe recessive, dominant, and intermediate osteopetrosis. J Bone Miner Res 18:1740-1747
76. 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
77. Fuchs R, Ellinger A, Pavelka M, Mellman I, Klapper H (1994) Rat liver endocytic coated vesicles do not exhibit ATP-dependent acidification in vitro. Proc Natl Acad Sci USA 91:4811-4815
78. Furman RE, Barchi RL (1978) The pathophysiology of myotonia produced by aromatic carboxylic acids. Ann Neurol 4:357-365
79. 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 Cell Physiol 274:C500-C512
80. 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
81. Gentzsch M, Cui L, Mengos A, Chang XB, Chen JH, Riordan JR (2003) The PDZ-binding chloride channel ClC-3B localizes to the Golgi and associates with cystic fibrosis transmembrane conductance regulatorinteracting PDZ proteins. J Biol Chem 278:6440-6449
82. Gill JR Jr, Bartter FC (1978) Evidence for a prostaglandin-independent defect in chloride reabsorption in the loop of Henle as a proximal cause of Bartter's syndrome. Am J Med 65:766-772
83. Gluck SL, Underhill DM, Iyori M, Holliday LS, Kostrominova TY, Lee BS (1996) Physiology and biochemistry of the kidney vacuolar H+-ATPase. Annu Rev Physiol 58:427-445
84. 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 ironlimited growth. Mol Gen Genet 241:542-553
85. Gründer S, Thiemann A, Pusch M, Jentsch TJ (1992) Regions involved in the opening of CIC-2 chloride channel by voltage and cell volume. Nature 360:759-762
86. Guggino WB (2004) The cystic fibrosis transmembrane regulator forms macromolecular complexes with PDZ domain scaffold proteins. Proc Am Thorac Soc 1:28-32
87. Günther W, Luchow A, Cluzeaud F, Vandewalle A, Jentsch TJ (1998) ClC-5, the chloride channel mutated in Dent's disease, colocalizes with the proton pump in endocytotically active kidney cells. Proc Natl Acad Sci USA 95:8075-8080
88. Günther W, Piwon N, Jentsch TJ (2003) The ClC-5 chloride channel knock-out mouse-an animal model for Dent's disease. Pflügers Arch 445:456-462
89. 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
90. Gyömörey K, Yeger H, Ackerley C, Garami E, Bear CE (2000) Expression of the chloride channel ClC-2 in the murine small intestine epithelium. Am J Physiol Cell Physiol 279:C1787-C1794
91. Hanke W, Miller C (1983) Single chloride channels from Torpedo electroplax. Activation by protons. J Gen Physiol 82:25-45
92. Hanrahan JW,Wioland MA (2004) Revisiting cystic fibrosis transmembrane conductance regulator structure and function. Proc Am Thorac Soc 1:17-21
93. Hansson JH, Nelson-Williams C, Suzuki H, Schild L, Shimkets R, Lu Y, Canessa C, Iwasaki T, Rossier B, Lifton RP (1995a) Hypertension caused by a truncated epithelial sodium channel gamma subunit:gEnetic heterogeneity of Liddle syndrome. Nat Genet 11:76-82
94. Hansson JH, Schild L, Lu Y, Wilson TA, Gautschi I, Shimkets R, Nelson-Williams C, Rossier BC, Lifton RP (1995b) A de novo missense mutation of the beta subunit of the epithelial sodium channel causes hypertension and Liddle syndrome, identifying a proline-rich segment critical for regulation of channel activity. Proc Natl Acad Sci USA 92:11495-11499
95. Hara-Chikuma M, Wang Y, Guggino SE, Guggino WB, Verkman AS (2005a) Impaired acidification in early endosomes of ClC-5 deficient proximal tubule. Biochem Biophys Res Commun 329:941-946
96. Hara-Chikuma M, Yang B, Sonawane ND, Sasaki S, Uchida S, Verkman AS (2005b) ClC-3 chloride channels facilitate endosomal acidification and chloride accumulation. J Biol Chem 280:1241-1247
97. Hartzell C, Putzier I, Arreola J (2005) Calcium-activated chloride channels. Annu Rev Physiol 67:719-758
98. Haug K, Warnstedt M, Alekov AK, Sander T, Ramirez A, Poser B, Maljevic S, Hebeisen S, Kubisch C, Rebstock J, Horvath S, Hallmann K, Dullinger JS, Rau B, Haverkamp F, Beyenburg S, Schulz H, Janz D, Giese B, Muller-Newen G, Propping P, Elger CE, Fahlke C, Lerche H, Heils A (2003) Mutations in CLCN2 encoding a voltage-gated chloride channel are associated with idiopathic generalized epilepsies. Nat Genet 33:527-532
99. Hayama A, Rai T, Sasaki S, Uchida S (2003) Molecular mechanisms of Bartter syndrome caused by mutations in the BSND gene. Histochem Cell Biol 119:485-493
100. 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 271:33632-33638
101. Hille B (2001) Ion channels of excitable membranes. Sinauer, Sunderland, MA
102. Hinzpeter A, Lipecka J, Brouillard F, Baudoin-Legros M, Dadlez M, Edelman A, Fritsch J (2006) Association between Hsp90 and the ClC-2 chloride channel upregulates channel function. Am J Physiol Cell Physiol 290:C45-C56.
103. Holmes KW, Hales R, Chu S, Maxwell MJ, Mogayzel PJ Jr, Zeitlin PL (2003) Modulation of Sp1 and Sp3 in lung epithelial cells regulates ClC-2 chloride channel expression. Am J Respir CellMol Biol 29:499-505
104. + exchange regulatory factor-2 with ClC-5. J Biol Chem 281:16068-16077
105. Hryciw DH, Ekberg J, Lee A, Lensink IL, Kumar S, Guggino WB, Cook DI, Pollock CA, Poronnik P (2004) Nedd4-2 functionally interacts with ClC-5:iNvolvement in constitutive albumin endocytosis in proximal tubule cells. J Biol Chem 279:54996-55007
106. Hryciw DH, Ekberg J, Pollock CA, Poronnik P (2005) ClC-5:A chloride channel with multiple roles in renal tubular albumin uptake. Int J Biochem Cell Biol 3:3
107. Hryciw DH, Wang Y, Devuyst O, Pollock CA, Poronnik P, Guggino WB (2003) Cofilin interacts with ClC-5 and regulates albumin uptake in proximal tubule cell lines. J Biol Chem 278:40169-40176
108. Huber S, Braun G, Schroppel B, Horster M (1998) Chloride channels ClC-2 and ICln mRNA expression differs in renal epithelial ontogeny. Kidney Int Suppl 67:S149-S151
109. Hübner CA, Stein V, Hermans-Borgmeyer I, Meyer T, Ballanyi K, Jentsch TJ (2001) Disruption of KCC2 reveals an essential role of K-Cl cotransport already in early synaptic inhibition. Neuron 30:515-524
110. Hunte C, Screpanti E, Venturi M, Rimon A, Padan E, Michel H (2005) Structure of a Na+/H+ antiporter and insights into mechanism of action and regulation by pH. Nature 435:1197-1202
111. Hunter M (2001) Accessory to kidney disease. Nature 414:502-503
112. Hutter OF,Warner AE (1967) Action of some foreign cations and anions on the chloride permeability of frog muscle. J Physiol 189:445-460
113. Iyer R, Iverson TM, Accardi A, Miller C (2002) A biological role for prokaryotic ClC chloride channels. Nature 419:715-718
114. Jeck N, Waldegger P, Doroszewicz J, Seyberth H, Waldegger S (2004a) A common sequence variation of the CLCNKB gene strongly activates ClC-Kb chloride channel activity. Kidney Int 65:190-197
115. Jeck N, Waldegger S, Lampert A, Boehmer C, Waldegger P, Lang PA, Wissinger B, Friedrich B, Risler T, Moehle R, Lang UE, Zill P, Bondy B, Schaeffeler E, Asante-Poku S, Seyberth H, Schwab M, Lang F (2004b) Activating mutation of the renal epithelial chloride channel ClC-Kb predisposing to hypertension. Hypertension 43:1175-1181
116. Jentsch TJ (2000) Neuronal KCNQ potassium channels:pHysiology and role in disease. Nat Rev Neurosci 1:21-30
117. Jentsch TJ (2005) Chloride transport in the kidney:lEssons from human disease and knockout mice. J Am Soc Nephrol 16:1549-1561
118. Jentsch TJ, Friedrich T, Schriever A, Yamada H (1999) The CLC chloride channel family. Pflügers Arch 437:783-795
119. Jentsch TJ, Maritzen T, Zdebik AA (2005a) Chloride channel diseases resulting from impaired transepithelial transport or vesicular function. J Clin Invest 115:2039-2046
120. Jentsch TJ, Neagoe I, Scheel O (2005b) CLC chloride channels and transporters. Curr Opin Neurobiol 15:319-325
121. Jentsch TJ, Poet M, Fuhrmann JC, Zdebik AA (2005c) Physiological functions of CLC Cl channels gleaned from human genetic disease and mouse models. Annu Rev Physiol 67:779-807
122. Jentsch TJ, Stein V, Weinreich F, Zdebik AA (2002) Molecular structure and physiological function of chloride channels. Physiol Rev 82:503-568
123. 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
124. Jordt SE, Jentsch TJ (1997) Molecular dissection of gating in the ClC-2 chloride channel. EMBO J 16:1582-1592
125. Karsdal MA, Henriksen K, Sørensen MG, Gram J, Schaller S, Dziegiel MH, Heegaard AM, Christophersen P, Martin TJ, Christiansen C, Bollerslev J (2005) Acidification of the osteoclastic resorption compartment provides insight into the coupling of bone formation to bone resorption. Am J Pathol 166:467-476
126. Kasper D, Planells-Cases R, Fuhrmann JC, Scheel O, Zeitz O, Ruether K, Schmitt A, Poet M, Steinfeld R, Schweizer M, Kornak U, Jentsch TJ (2005) Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration. EMBO J 24:1079-1091
127. Kawasaki M, Uchida S, Monkawa T, Miyawaki A, Mikoshiba K, Marumo F, Sasaki S (1994) Cloning and expression of a protein kinase C-regulated chloride channel abundantly expressed in rat brain neuronal cells. Neuron 12:597-604
128. Kibble JD, Trezise AE, Brown PD (1996) Properties of the cAMP-activated Cl- current in choroid plexus epithelial cells isolated from the rat. J Physiol 496:69-80
129. 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
130. Kobayashi K, Uchida S, Mizutani S, Sasaki S, Marumo F (2001) Developmental expression of CLC-K1 in the postnatal rat kidney. Histochem Cell Biol 116:49-56
131. Kokubo Y, Iwai N, Tago N, Inamoto N, Okayama A, Yamawaki H, Naraba H, Tomoike H (2005) Association analysis between hypertension and CYBA, CLCNKB, and KCNMB1 functional polymorphisms in the Japanese population - the Suita Study. Circ J 69:138-142
132. Konrad M, Vollmer M, Lemmink HH, van den Heuvel LP, Jeck N, Vargas-Poussou R, Lakings A, Ruf R, Deschenes G, Antignac C, Guay-Woodford L, Knoers NV, Seyberth HW, Feldmann D, Hildebrandt F (2000) Mutations in the chloride channel gene CLCNKB as a cause of classic Bartter syndrome. J Am Soc Nephrol 11:1449-1459
133. Kornak U, Kasper D, Bösl MR, Kaiser E, Schweizer M, Schulz A, Friedrich W, Delling G, Jentsch TJ (2001) Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man. Cell 104:205-215
134. Kornak U, Ostertag A, Branger S, Benichou O, de Vernejoul MC (2006) Polymorphisms in the CLCN7 gene modulate bone density in postmenopausal women and in patients with autosomal dominant osteopetrosis type II. J Clin Endocrinol Metab 91:995-1000
135. Kumar V, Farell G, Deganello S, Lieske JC (2003) Annexin II is present on renal epithelial cells and binds calcium oxalate monohydrate crystals. J Am Soc Nephrol 14:289-297
136. 2+ and other sulfhydryl-reactive compounds. Pflügers Arch 433:357-363
137. Lange PF, Wartosch L, Jentsch TJ, Fuhrmann JC (2006) ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function. Nature 440:220-223
138. Lee JK, Kozono D, Remis J, Kitagawa Y, Agre P, Stroud RM (2005) Structural basis for conductance by the archaeal aquaporin AqpM at 1.68 Å. Proc Natl Acad Sci USA 102:18932-18937
139. Lehmann-Horn F, Jurkat-Rott K (1999) Voltage-gated ion channels and hereditary disease. Physiol Rev 79:1317-1372
140. Li X, Shimada K, Showalter LA, Weinman SA (2000) Biophysical properties of ClC-3 differentiate it from swelling-activated chloride channels in Chinese hamster ovary-K1 cells. J Biol Chem 275:35994-35998
141. 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-C1491
142. Liantonio A, Accardi A, Carbonara G, Fracchiolla G, Loiodice F, Tortorella P, Traverso S, Guida P, Pierno S, De Luca A, Camerino DC, Pusch M (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 62:265-271
143. Liantonio A, De Luca A, Pierno S, Didonna MP, Loiodice F, Fracchiolla G, Tortorella P, Antonio L, Bonerba E, Traverso S, Elia L, Picollo A, Pusch M, Conte Camerino D (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 139:1255-1264
144. Liantonio A, Picollo A, Babini E, Carbonara G, Fracchiolla G, Loiodice F, Tortorella V, Pusch M, Camerino DC (2006) Activation and inhibition of kidney CLC-K chloride channels by fenamates. Mol Pharmacol 69:165-173
145. Liantonio A, Pusch M, Picollo A, Guida P, De Luca A, Pierno S, Fracchiolla G, Loiodice F, Tortorella P, Conte Camerino D (2004) Investigations of pharmacologic properties of the renal CLC-K1 chloride channel co-expressed with barttin by the use of 2-(p-chlorophenoxy)propionic acid derivatives and other structurally unrelated chloride channel blockers. J Am Soc Nephrol 15:13-20
146. 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
147. Lipecka J, Bali M, Thomas A, Fanen P, Edelman A, Fritsch J (2002) Distribution of ClC-2 chloride channel in rat and human epithelial tissues. Am J Physiol Cell Physiol 282:C805-C816
148. Liu W, Morimoto T, Kondo Y, Iinuma K, Uchida S, Imai M (2001) "Avian-type" renal medullary tubule organization causes immaturity of urine-concentrating ability in neonates. Kidney Int 60:680-693
149. Lobet S, Dutzler R (2006) Ion-binding properties of the ClC chloride selectivity filter. EMBO J 25:24-33
150. Lorenz C, Pusch M, Jentsch TJ (1996) Heteromultimeric CLC chloride channels with novel properties. Proc Natl Acad Sci USA 93:13362-13366
151. Ludewig U, Jentsch TJ, Pusch M (1997a) Analysis of a protein region involved in permeation and gating of the voltage-gated Torpedo chloride channel ClC-0. J Physiol 498:691-702
152. Ludewig U, Pusch M, Jentsch TJ (1996) Two physically distinct pores in the dimeric ClC-0 chloride channel. Nature 383:340-343
153. Ludewig U, Pusch M, Jentsch TJ (1997b) Independent gating of single pores in CLC-0 chloride channels. Biophys J 73:789-797
154. Ludwig M, Doroszewicz J, Seyberth HW, Bokenkamp A, Balluch B, Nuutinen M, Utsch B, Waldegger S (2005) Functional evaluation of Dent's disease-causing mutations:iMplications for ClC-5 channel traf- ficking and internalization. Hum Genet 117:228-237
155. Ludwig M, Waldegger S, Nuutinen M, Bokenkamp A, Reissinger A, Steckelbroeck S, Utsch B (2003) Four additional CLCN5 exons encode a widely expressed novel long CLC-5 isoform but fail to explain Dent's phenotype in patients without mutations in the short variant. Kidney Blood Press Res 26:176-184
156. Maack T, Park CH (1990) Endocytosis and lysosomal hydrolysis of proteins in proximal tubules. Methods Enzymol 191:340-354
157. Maduke M, Miller C, Mindell JA (2000) A decade of CLC chloride channels:sTructure, mechanism, and many unsettled questions. Annu Rev Biophys Biomol Struct 29:411-438
158. 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 114:713-722
159. Maduke M, Williams C, Miller C (1998) Formation of CLC-0 chloride channels from separated transmembrane and cytoplasmic domains. Biochemistry 37:1315-1321
160. Malinowska DH, Kupert EY, Bahinski A, Sherry AM, Cuppoletti J (1995) Cloning, functional expression, and characterization of a PKA-activated gastric Cl- channel. Am J Physiol Cell Physiol 268:C191-C200
161. Mankodi A, Takahashi MP, Jiang H, Beck CL, Bowers WJ, Moxley RT, Cannon SC, Thornton CA (2002) Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy. Mol Cell 10:35-44
162. Maranda B, Brown D, Bourgoin S, Casanova JE, Vinay P, Ausiello DA, Marshansky V (2001) Intraendosomal pH-sensitive recruitment of the Arf-nucleotide exchange factor ARNO and Arf6 from cytoplasm to proximal tubule endosomes. J Biol Chem 276:18540-18550
163. Marty A, Llano I (2005) Excitatory effects of GABA in established brain networks. Trends Neurosci 28:284-289
164. Matsumura Y, Uchida S, Kondo Y, Miyazaki H, Ko SB, Hayama A, Morimoto T, LiuW, Arisawa M, Sasaki S, Marumo F (1999) Overt nephrogenic diabetes insipidus in mice lacking the CLC-K1 chloride channel. Nat Genet 21:95-98
165. Mehrke G, Brinkmeier H, Jockusch H (1988) The myotonic mouse mutant ADR:eLectrophysiology of the muscle fiber. Muscle Nerve 11:440-446
166. Mellman I (1996) Endocytosis and molecular sorting. Annu Rev Dev Biol 12:575-625
167. Mellman I, Fuchs R, Helenius A (1986) Acidification of the endocytic and exocytic pathways. Annu Rev Biochem 55:663-700
168. Meyer S, Dutzler R (2006) Crystal structure of the cytoplasmic domain of the chloride channel ClC-0. Structure 14:299-307
169. Meyer-Kleine C, Steinmeyer K, Ricker K, Jentsch TJ, Koch MC (1995) Spectrum of mutations in the major human skeletal muscle chloride channel gene (CLCN1) leading to myotonia. Am J Hum Genet 57:1325-1334
170. Middleton RE, Pheasant DJ, Miller C (1996) Homodimeric architecture of a ClC-type chloride ion channel. Nature 383:337-340
171. Miller C (1982) Open-state substructure of single chloride channels from Torpedo electroplax. Philos Trans R Soc Lond B Biol Sci 299:401-411
172. Miller C (2006) ClC chloride channels viewed through a transporter lens. Nature 440:484-489
173. Miller C, Richard EA (1990) 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. F.J. Alvarez-Leefmans and J.M. Russell, editors.
174. Miller C, White MM (1980) A voltage-dependent chloride conductance channel from Torpedo electroplax membrane. Ann NY Acad Sci 341:534-551
175. Miller C, White MM (1984) Dimeric structure of single chloride channels from Torpedo electroplax. Proc Natl Acad Sci USA 81:2772-2775
176. Miller MD, Schwarzenbacher R, von Delft F, Abdubek P, Ambing E, Biorac T, Brinen LS, Canaves JM, Cambell J, Chiu HJ, Dai X, Deacon AM, DiDonato M, Elsliger MA, Eshagi S, Floyd R, Godzik A, Grittini C, Grzechnik SK, Hampton E, Jaroszewski L, Karlak C, Klock HE, Koesema E, Kovarik JS, Kreusch A, Kuhn P, Lesley SA, Levin I, McMullan D, McPhillips TM, Morse A, Moy K, Ouyang J, Page R, Quijano K, Robb A, Spraggon G, Stevens RC, van den Bedem H, Velasquez J, Vincent J, Wang X, West B, Wolf G, Xu Q, Hodgson KO, Wooley J, Wilson IA (2004) Crystal structure of a tandem cystathionine-beta-synthase (CBS) domain protein (TM0935) from Thermotoga maritima at 1.87 Å resolution. Proteins 57:213-217
177. Mindell JA, Maduke M (2001) ClC chloride channels. Genome Biol 2:REVIEWS3003
178. Mindell JA, Maduke M, Miller C, Grigorieff N (2001) Projection structure of a ClC-type chloride channel at 6.5 Å resolution. Nature 409:219-223
179. Misgeld U, Deisz RA, Dodt HU, Lux HD (1986) The role of chloride transport in postsynaptic inhibition of hippocampal neurons. Science 232:1413-1415
180. Miyazawa A, Fujiyoshi Y, Unwin N (2003) Structure and gating mechanism of the acetylcholine receptor pore. Nature 423:949-955
181. Mo L, Wills NK (2004) ClC-5 chloride channel alters expression of the epithelial sodium channel (ENaC). J Membr Biol 202:21-37
182. Mo L, Xiong W, Qian T, Sun H, Wills NK (2004) Coexpression of complementary fragments of ClC-5 and restoration of chloride channel function in a Dent's disease mutation. Am J Physiol Cell Physiol 286:C79-C89
183. Mohammad-Panah R, Harrison R, Dhani S, Ackerley C, Huan LJ, Wang Y, Bear CE (2003) The chloride channel ClC-4 contributes to endosomal acidification and trafficking. J Biol Chem 278:29267-29277
184. Moreland JG, Davis AP, Bailey G, Nauseef WM, Lamb FS (2006) Anion channels including CLC-3 are required for normal neutrophil oxidative function, phagocytosis, and transendothelial migration. J Biol Chem 7:7
185. +-ATPase without ultrastructural changes in kidneys of Dent's disease patients. Kidney Int 63:1285-1295
186. Murata K, Mitsuoka K, Hirai T, Walz T, Agre P, Heymann JB, Engel A, Fujiyoshi Y (2000) Structural determinants of water permeation through aquaporin-1. Nature 407:599-605
187. Murray CB, Chu S, Zeitlin PL (1996) Gestational and tissue-specific regulation of C1C-2 chloride channel expression. Am J Physiol Lung Cell Mol Physiol 271:L829-L837
188. Murray CB, Morales MM, Flotte TR, McGrath-Morrow SA, Guggino WB, Zeitlin PL (1995) CIC-2:a developmentally dependent chloride channel expressed in the fetal lung and downregulated after birth. Am J Respir Cell Mol Biol 12:597-604
189. Nakatsu F, Ohno H (2003) Adaptor protein complexes as the key regulators of protein sorting in the post- Golgi network. Cell Struct Funct 28:419-429
190. Nascimento DS, Reis CU, Goldenberg RC, Ortiga-Carvalho TM, Pazos-Moura CC, Guggino SE, GugginoWB, Morales MM (2003) Estrogen modulates ClC-2 chloride channel gene expression in rat kidney. Plügers Arch 446:593-599
191. Niemeyer MI, Cid LP, Zúñiga L, Catalán M, Sepülveda FV (2003) A conserved pore-lining glutamate as a voltage- and chloride-dependent gate in the ClC-2 chloride channel. J Physiol 553:873-879
192. Niemeyer MI, Yusef YR, Cornejo I, Flores CA, Sepúlveda FV, Cid LP (2004) Functional evaluation of human CIC-2 chloride channel mutations associated with idiopathic generalized epilepsies. Physiol Genomics 19:74-83
193. Cl(swell). Am J Physiol Cell Physiol 287:C1426-C1435
194. Ogura T, Furukawa T, Toyozaki T, Yamada K, Zheng YJ, Katayama Y, Nakaya H, Inagaki N (2002) CIC-3B, a novel CIC-3 splicing variant that interacts with EBP50 and facilitates expression of CFTR-regulated ORCC. FASEB J 16:863-865
195. Palade PT, Barchi RL (1977) Characteristics of the chloride conductance in muscle fibers of the rat diaphragm. J Gen Physiol 69:325-342
196. Papponen H, Kaisto T, Myllyla VV, Myllyla R, Metsikko K (2005) Regulated sarcolemmal localization of the muscle-specific CIC-1 chloride channel. Exp Neurol 191:163-173
197. Peña-Münzenmayer G, Catalän M, Cornejo I, Figueroa CD, Melvin JE, Niemeyer MI, Cid LP, Sepúlveda FV (2005) Basolateral localization of native CIC-2 chloride channels in absorptive intestinal epithelial cells and basolateral sorting encoded by a CBS-2 domain di-leucine motif. J Cell Sci 118:4243-4252
198. Picollo A, Liantonio A, Didonna MP, Elia L, Camerino DC, Pusch M (2004) Molecular determinants of differential pore blocking of kidney CLC-K chloride channels. EMBO Rep 5:584-589
199. Picollo A, Pusch M(2005) Chloride/proton antiporter activity of mammalian CLC proteins ClC-4 and ClC-5. Nature 436:420-423
200. --channel disruption impairs endocytosis in a mouse model for Dent's disease. Nature 408:369-373
201. 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
202. Ponting CP (1997) CBS domains in CIC chloride channels implicated inmyotonia and nephrolithiasis (kidney stones). J Mol Med 75:160-163
203. Pusch M (1996) Knocking on channel's door. The permeating chloride ion acts as the gating charge in CIC-0. J Gen Physiol 108:233-236
204. Pusch M (2001) Chloride channelopathies. Pharmaceutical News 8:45-51
205. Pusch M (2002) Myotonia caused by mutations in the muscle chloride channel gene CLCN1. Hum Mutat 19:423-434
206. Pusch M, Accardi A, Liantonio A, Ferrera L, De Luca A, Camerino DC, Conti F (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
207. Pusch M, Jordt SE, Stein V, Jentsch TJ (1999) Chloride dependence of hyperpolarization-activated chloride channel gates. J Physiol 515:341-353
208. Pusch M, Liantonio A, Bertorello L, Accardi A, De Luca A, Pierno S, Tortorella V, Camerino DC (2000) Pharmacological characterization of chloride channels belonging to the ClC family by the use of chiral clofibric acid derivatives. Mol Pharmacol 58:498-507
209. Pusch M, Ludewig U, Jentsch TJ (1997) Temperature dependence of fast and slow gating relaxations of CIC-0 chloride channels. J Gen Physiol 109:105-116
210. Pusch M, Ludewig U, Rehfeldt A, Jentsch TJ (1995a) Gating of the voltage-dependent chloride channel CIC-0 by the permeant anion. Nature 373:527-531
211. 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
212. Pusch M, Steinmeyer K, Koch MC, Jentsch TJ (1995b) Mutations in dominant human myotonia congenita drastically alter the voltage dependence of the ClC-1 chloride channel. Neuron 15:1455-1463
213. Pusch M, Zifarelli G, Murgia AR, Picollo A, Babini E (2006) Channel or transporter? The CLC saga continues. Exp Physiol 91:149-152.
214. Qualmann B, Kessels MM, Kelly RB (2000) Molecular links between endocytosis and the actin cytoskeleton. J Cell Biol 150:F111-F116.
215. Ramjeesingh M, Li C, She YM, Bear CE (2006) Evaluation of the membrane domain of CLC-2. Biochem J 9:9
216. Richard EA, Miller C (1990) Steady-state coupling of ion-channel conformations to a transmembrane ion gradient. Science 247:1208-1210
217. Riordan JR (2005) Assembly of functional CFTR chloride channels. Annu Rev Physiol 67:701-718
218. Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou JL, Drumm ML, Iannuzzi MC, Collins FS, Tsui L-C (1989) Identification of the cystic fibrosis gene:cLoning and characterization of complementary DNA. Science 245:1066-1073
219. Robinson NC, Huang P, Kaetzel MA, Lamb FS, Nelson DJ (2004) Identification of an N-terminal amino acid of the CLC-3 chloride channel critical in phosphorylation-dependent activation of a CaMKII-activated chloride current. J Physiol 556:353-368
220. 2+ channels in retinal pigment epithelial cells. FASEB J 20:178-180.
221. + channel:eLectrostatic stabilization of monovalent cations. Science 285:100-102
222. Rychkov G, Pusch M, Roberts M, Bretag A (2001) Interaction of hydrophobic anions with the rat skeletal muscle chloride channel CIC-1:eFfects on permeation and gating. J Physiol 530:379-393
223. 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 501:355-362
224. 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 497:423-435
225. 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 111:653-665
226. Salas-Casas A, Ponce-Balderas A, Garcia-Perez RM, Cortes-Reynosa P, Gamba G, Orozco E, Rodriguez MA (2006) Identification and functional characterization of EhlIC-A, an Entamoeba histolytica CIC chloride channel located at plasma membrane. Mol Microbiol 59:1249-1261
227. Salazar G, Love R, Styers ML,Werner E, Peden A, Rodriguez S, Gearing M, Wainer BH, Faundez V (2004) AP-3-dependent mechanisms control the targeting of a chloride channel (CIC-3) in neuronal and nonneuronal cells. J Biol Chem 279:25430-25439
228. Sands JM, Bichet DG (2006) Nephrogenic diabetes insipidus. Ann Intern Med 144:186-194
229. Santos Ornellas D, Grozovsky R, Goldenberg RC, Carvalho DP, Fong P, Guggino WB, Morales M (2003) Thyroid hormone modulates CIC-2 chloride channel gene expression in rat renal proximal tubules. J Endocrinol 178:503-511
230. 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 113:457-468
231. Schaller S, Henriksen K, Sveigaard C, Heegaard AM, Helix N, Stahlhut M, Ovejero MC, Johansen JV, Solberg H, Andersen TL, Hougaard D, Berryman M, Shiodt CB, Sorensen BH, Lichtenberg J, Christophersen P, Foged NT, Delaisse JM, Engsig MT, Karsdal MA (2004) The chloride channel inhibitor NS3736 [corrected] prevents bone resorption in ovariectomized rats without changing bone formation. J Bone Miner Res 19:1144-1153
232. Scheel O, Zdebik AA, Lourdel S, Jentsch TJ (2005) Voltage-dependent electrogenic chloride/proton exchange by endosomal CLC proteins. Nature 436:424-427
233. Schlingmann KP, Konrad M, Jeck N, Waldegger P, Reinalter SC, Holder M, Seyberth HW, Waldegger S (2004) Salt wasting and deafness resulting from mutations in two chloride channels. N Engl J Med 350:1314-1319
234. 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
235. - channel mutated in Dent's disease. J Biol Chem 276:12049-12054
236. 2 and COOH terminus of the yeast CLC putative chloride channel Gef1p. J Biol Chem 273:15110-15118
237. Scott JW, Hawley SA, Green KA, Anis M, Stewart G, Scullion GA, Norman DG, Hardie DG (2004) CBS domains form energy-sensing modules whose binding of adenosine ligands is disrupted by disease mutations. J Clin Invest 113:274-284
238. Silva IV, Cebotaru V, Wang H, Wang XT, Wang SS, Guo G, Devuyst O, Thakker RV, Guggino WB, Guggino SE (2003) The ClC-5 knockout mouse model of Dent's disease has renal hypercalciuria and increased bone turnover. J Bone Miner Res 18:615-623
239. 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. Nat Genet 17:171-178
240. Sintchak MD, Fleming MA, Futer O, Raybuck SA, Chambers SP, Caron PR, Murcko MA,Wilson KP (1996) Structure and mechanism of inosine monophosphate dehydrogenase in complex with the immunosuppressant mycophenolic acid. Cell 85:921-930
241. 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
242. Sobacchi C, Frattini A, Orchard P, Porras O, Tezcan I, Andolina M, Babul-Hirji R, Baric I, Canham N, Chitayat D, Dupuis-Girod S, Ellis I, Etzioni A, Fasth A, Fisher A, Gerritsen B, Gulino V, Horwitz E, Klamroth V, Lanino E, Mirolo M, Musio A, Matthijs G, Nonomaya S, Notarangelo LD, Ochs HD, Superti Furga A, Valiaho J, van Hove JL, Vihinen M, Vujic D, Vezzoni P, Villa A (2001) The mutational spectrum of human malignant autosomal recessive osteopetrosis. Hum Mol Genet 10:1767-1773
243. Speake T, Kajita H, Smith CP, Brown PD (2002) Inward-rectifying anion channels are expressed in the epithelial cells of choroid plexus isolated from ClC-2 'knock-out' mice. J Physiol 539:385-390
244. Speirs HJ, Wang WY, Benjafield AV, Morris BJ (2005) No association with hypertension of CLCNKB and TNFRSF1B polymorphisms at a hypertension locus on chromosome 1p36. J Hypertens 23:1491-1496
245. A receptor function following gene transfer of the CLC-2 chloride channel. Neuron 17:543-551
246. Steinmeyer K, Klocke R, Ortland C, Gronemeier M, Jockusch H, Gründer S, Jentsch TJ (1991a) Inactivation of muscle chloride channel by transposon insertion in myotonic mice. Nature 354:304-308
247. Steinmeyer K, Lorenz C, Pusch M, Koch MC, Jentsch TJ (1994) Multimeric structure of CIC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen). EMBO J 13:737-743
248. Steinmeyer K, Ortland C, Jentsch TJ (1991b) Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel. Nature 354:301-304
249. 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
250. Stobrawa SM, Breiderhoff T, Takamori S, Engel D, Schweizer M, Zdebik AA, Bösl MR, Ruether K, Jahn H, Draguhn A, Jahn R, Jentsch TJ (2001) Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus. Neuron 29:185-196
251. Strange K (2003) From genes to integrative physiology:iOn channel and transporter biology in Caenorhabditis elegans. Physiol Rev 83:377-415
252. Suzuki T, Rai T, Hayama A, Sohara E, Suda S, Itoh T, Sasaki S, Uchida S (2006) Intracellular localization of CIC chloride channels and their ability to form hetero-oligomers. J Cell Physiol 206:792-798
253. Tanford C (1983) Mechanism of free energy coupling in active transport. Ann Rev Biochem 52:379-409
254. Thiemann A, Gründer S, Pusch M, Jentsch TJ (1992) A chloride channel widely expressed in epithelial and non-epithelial cells. Nature 356:57-60
255. Thompson CH, Fields DM, Olivetti PR, Fuller MD, Zhang ZR, Kubanek J, McCarty NA (2005) Inhibition of ClC-2 chloride channels by a peptide component or components of scorpion venom. J Membr Biol 208:65-76.
256. Thomsen J (1876) Tonische Krämpfe in willkürlich beweglichen Muskeln in Folge von ererbter psychischer Disposition. Arch Psychatrie Nerv 6:702-718
257. Toyoshima C, Nakasako M, Nomura H, Ogawa H (2000) Crystal structure of the calcium pump of sarcoplasmic reticulum at 2.6 Å resolution. Nature 405:647-655
258. 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
259. Traverso S, Zifarelli G, Aiello R, Pusch M (2006) Proton sensing of CLC-0 mutant E166D. J Gen Physiol 127:51-66
260. Tsui LC (1991) Probing the basic defect in cystic fibrosis. Curr Opin Genet Dev 1:4-10
261. Tyteca D, Van Der Smissen P, Mettlen M, Van Bambeke F, Tulkens PM, Mingeot-Leclercq MP, Courtoy PJ (2002) Azithromycin, a lysosomotropic antibiotic, has distinct effects on fluid-phase and receptor-mediated endocytosis, but does not impair phagocytosis in J774 macrophages. Exp Cell Res 281:86-100
262. Uchida S, Sasaki S (2005) Function of chloride channels in the kidney. Annu Rev Physiol 67:759-778
263. 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
264. 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, CIC-K1. J Clin Invest 95:104-113
265. van den Hove MF, Croizet-Berger K, Jouret F, Guggino SE, Guggino WB, Devuyst O, Courtoy PJ (2006) The loss of the chloride channel, ClC-5, delays apical iodide efflux and induces a euthyroid goiter in the mouse thyroid gland. Endocrinology 147:1287-1296
266. van Slegtenhorst MA, Bassi MT, Borsani G, Wapenaar MC, Ferrero GB, de Conciliis L, Rugarli EI, Grillo A, Franco B, Zoghbi HY, et al. (1994) A gene from the Xp22.3 region shares homology with voltage-gated chloride channels. Hum Mol Genet 3:547-552
267. 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 Renal Physiol 272:F678-F688
268. Vandewalle A, Cluzeaud F, Peng KC, Bens M, Lüchow A, Günther W, Jentsch TJ (2001) Tissue distribution and subcellular localization of the ClC-5 chloride channel in rat intestinal cells. Am J Physiol Cell Physiol 280:C373-C381
269. Varela D, Niemeyer MI, Cid LP, Sepúlveda FV (2002) Effect of an N-terminus deletion on voltage-dependent gating of the ClC-2 chloride channel. J Physiol 544:363-372
270. Vij N, Zeitlin PL (2006) Regulation of the ClC-2 lung epithelial chloride channel by glycosylation of SP1. Am J Respir Cell Mol Biol 34:754-759
271. Vogt K, Mellor J, Tong G, Nicoll R (2000) The actions of synaptically released zinc at hippocampal mossy fiber synapses. Neuron 26:187-196
272. Waldegger S, Jeck N, Barth P, Peters M, Vitzthum H, Wolf K, Kurtz A, Konrad M, Seyberth HW (2002) Barttin increases surface expression and changes current properties of ClC-K channels. Pflügers Arch 444:411-418
273. Waldegger S, Jentsch TJ (2000) Functional and structural analysis of ClC-K chloride channels involved in renal disease. J Biol Chem 275:24527-24533
274. Wang J, Xu H, Morishima S, Tanabe S, Jishage K, Uchida S, Sasaki S, Okada Y, Shimizu T (2006) Single-channel properties of volume-sensitive Cl- channel in ClC-3-deficient cardiomyocytes. Jpn J Physiol 31:31
275. Wang SS, Devuyst O, Courtoy PJ, Wang XT, Wang H, Wang Y, Thakker RV, Guggino S, Guggino WB (2000) Mice lacking renal chloride channel, CLC-5, are a model for Dent's disease, a nephrolithiasis disorder associated with defective receptor-mediated endocytosis. Hum Mol Genet 9:2937-2945
276. Wang T, Weinman SA (2004) Involvement of chloride channels in hepatic copper metabolism: CIC-4 promotes copper incorporation into ceruloplasmin. Gastroenterology 126:1157-1166
277. Wang Y, Cai H, Cebotaru L, Hryciw DH, Weinman EJ, Donowitz M, Guggino SE, Guggino WB (2005) ClC-5:rOle in endocytosis in the proximal tubule. Am J Physiol Renal Physiol 289:F850-F862
278. Weinreich F, Jentsch TJ (2001) Pores formed by single subunits in mixed dimers of different CLC chloride channels. J Biol Chem 276:2347-2353
279. Wellhauser L, Kuo HH, Stratford FL, Ramjeesingh M, Huan LJ, Luong W, Li C, Deber CM, Bear CE (2006) Nucleotides bind to the carboxy terminus of ClC-5. Biochem J 11:11
280. Weng TX, Godley BF, Jin GF, Mangini NJ, Kennedy BG, Yu AS, Wills NK (2002) Oxidant and antioxidant modulation of chloride channels expressed in human retinal pigment epithelium. Am J Physiol Cell Physiol 283:C839-C849
281. White MM, Miller C (1979) A voltage-gated anion channel from the electric organ of Torpedo californica. J Biol Chem 254:10161-10166
282. Wohlke A, Distl O, Drogemuller C (2006) Characterization of the canine CLCN3 gene and evaluation as candidate for late-onset NCL. BMC Genet 7:13
283. 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
284. Wotring VE, Miller TS, Weiss DS (2003) Mutations at the GABA receptor selectivity filter:a possible role for effective charges. J Physiol 548:527-540
285. Wright EM, Diamond JM (1977) Anion selectivity in biological systems. Physiol Rev 57:109-156
286. Wrong OM, Norden AG, Feest TG (1994) Dent's disease; a familial proximal renal tubular syndrome with low-molecular-weight proteinuria, hypercalciuria, nephrocalcinosis, metabolic bone disease, progressive renal failure and a marked male predominance. QJM 87:473-493
287. Yin J, Kuang Z, Mahankali U, Beck TL (2004) Ion transit pathways and gating in CIC chloride channels. Proteins 57:414-421
288. Yoshikawa M, Uchida S, Ezaki J, Rai T, Hayama A, Kobayashi K, Kida Y, NodaM, KoikeM, Uchiyama Y, Marumo F, Kominami E, Sasaki S (2002) CLC-3 deficiency leads to phenotypes similar to human neuronal ceroid lipofuscinosis. Genes Cells 7:597-605
289. Yusef YR, Zúñiga L, Catalán M, Niemeyer MI, Cid LP, Sepúlveda FV (2006) Removal of gating in voltage-dependent ClC-2 chloride channel by point mutations affecting the pore and C-terminus CBS-2 domain. J Physiol 9:9
290. Zdebik AA, Cuffe JE, Bertog M, Korbmacher C, Jentsch TJ (2004) Additional disruption of the CIC-2 CI-channel does not exacerbate the cystic fibrosis phenotype of cystic fibrosis transmembrane conductance regulator mouse models. J Biol Chem 279:22276-22283
291. Zhang R, Evans G, Rotella FJ, Westbrook EM, Beno D, Huberman E, Joachimiak A, Collart FR (1999) Characteristics and crystal structure of bacterial inosine-5′-monophosphate dehydrogenase. Biochemistry 38:4691-4700
292. Zhang XD, Li Y, Yu WP, Chen TY (2006) Roles of K149, G352, and H401 in the channel functions of ClC-0:TEsting the predictions from theoretical calculations. J Gen Physiol 127:435-447
293. + channel-Fab complex at 2.0 Å resolution. Nature 414:43-48
294. Zúñiga L, Niemeyer MI, Varela D, Catalán M, Cid LP, Sepúlveda FV (2004) The voltage-dependent CIC-2 chloride channel has a dual gating mechanism. J Physiol 555:671-682