Physiological Functions of the CLC Chloride Transport Proteins

Advances in Molecular and Cell Biology

Volumn 38, Issue , 2006, Pages 9-57

  Maritzen, Tanja ^a   Blanz, Judith ^a   Jentsch, Thomas ^a  

^a UNIVERSITY MEDICAL CENTER HAMBURG EPPENDORF   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

EID: 33846610602     PISSN: 15692558     EISSN: None     Source Type: Book Series    
DOI: 10.1016/S1569-2558(06)38002-2     Document Type: Chapter

References (217)

1. - channels. Nature 427 (2004) 803-807
2. Adachi S., Uchida S., Ito H., Hata M., Hiroe M., Marumo F., and Sasaki S. 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 (1994) 17677-17683
3. Adler D.A., Rugarli E.I., Lingenfelter P.A., Tsuchiya K., Poslinski D., Liggitt H.D., Chapman V.M., Elliott R.W., Ballabio A., and Disteche C.M. Evidence of evolutionary up-regulation of the single active X chromosome in mammals based on Clc4 expression levels in Mus spretus and Mus musculus. Proc. Natl. Acad. Sci. USA 94 (1997) 9244-9248
4. - currents. Neurosci. Lett. 284 (2000) 171-174
5. Aniento F., Gu F., Parton R.G., and Gruenberg J. An endosomal beta COP is involved in the pH-dependent formation of transport vesicles destined for late endosomes. J. Cell Biol. 133 (1996) 29-41
6. - channel gene. J. Physiol. 545 Pt. 1 (2002) 207-216
7. +/Pi cotransporter in mouse kidney. Pflügers Arch. 446 (2003) 52-60
8. Barbier-Brygoo H., Vinauger M., Colcombet J., Ephritikhine G., Frachisse J., and Maurel C. Anion channels in higher plants: Functional characterization, molecular structure and physiological role. Biochim. Biophys. Acta 1465 (2000) 199-218
9. - uptake and acidification. J. Cell Sci. 114 (2001) 2145-2154
10. Basu S.K., Goldstein J.L., Anderson R.G., and Brown M.S. Monensin interrupts the recycling of low density lipoprotein receptors in human fibroblasts. Cell 24 (1981) 493-502
11. Bayer M.J., Reese C., Buhler S., Peters C., and Mayer A. Vacuole membrane fusion: V0 functions after trans-SNARE pairing and is coupled to the Ca2+ -releasing channel. J. Cell Biol. 162 (2003) 211-222
12. Beck C.L., Fahlke C., and George Jr. A.L. Molecular basis for decreased muscle chloride conductance in the myotonic goat. Proc. Natl. Acad. Sci. USA 93 (1996) 11248-11252
13. Bellocchio E.E., Reimer R.J., Fremeau Jr. R.T., and Edwards R.H. Uptake of glutamate into synaptic vesicles by an inorganic phosphate transporter. Science 289 (2000) 957-960
14. Bennetts B., Rychkov G.Y., Ng H.L., Morton C.J., Stapleton D., Parker M.W., and Cromer B.A. Cytoplasmic ATP-sensing domains regulate gating of skeletal muscle ClC-1 chloride channels. J. Biol. Chem. 280 (2005) 32452-32458
15. Birkenhäger R., Otto E., Schürmann M.J., Vollmer M., Ruf E.M., Maier-Lutz I., Beekmann F., Fekete A., Omran H., Feldmann D., Milford D.V., Jeck N., et al. Mutation of BSND causes Bartter syndrome with sensorineural deafness and kidney failure. Nat. Genet. 29 (2001) 310-314
16. Blaisdell C.J., Edmonds R.D., Wang X.T., Guggino S., and Zeitlin P.L. pH-regulated chloride secretion in fetal lung epithelia. Am. J. Physiol. Lung Cell Mol. Physiol. 278 (2000) L1248-L1255
17. Borsani G., Rugarli E.I., Taglialatela M., Wong C., and Ballabio A. Characterization of a human and murine gene (CLCN3) sharing similarities to voltage-gated chloride channels and to a yeast integral membrane protein. Genomics 27 (1995) 131-141
18. --channel disruption. EMBO J. 20 (2001) 1289-1299
19. Böttger T., Hübner C., Maier H., Rust M.B., Beck F.X., and Jentsch T.J. Deafness and renal tubular acidosis in mice lacking the K-Cl cotransporter Kcc4. Nature 416 (2002) 874-878
20. Böttger T., Rust M.B., Maier H., Seidenbecher T., Schweizer M., Keating D.J., Faulhaber J., Ehmke H., Pfeffer C., Scheel O., Lemcke B., Horst J., et al. Loss of K-Cl co-transporter KCC3 causes deafness, neurodegeneration and reduced seizure threshold. EMBO J. 22 (2003) 5422-5434
21. Boyle W.J., Simonet W.S., and Lacey D.L. Osteoclast differentiation and activation. Nature 423 (2003) 337-342
22. Brandt S., and Jentsch T.J. ClC-6 and ClC-7 are two novel broadly expressed members of the CLC chloride channel family. FEBS Lett. 377 (1995) 15-20
23. Bretag A.H., Dawe S.R., and Moskwa A.G. Chemically induced myotonia in amphibia. Nature 286 (1980) 625-626
24. Bronsveld I., Mekus F., Bijman J., Ballmann M., de Jonge H.R., Laabs U., Halley D.J., Ellemunter H., Mastella G., Thomas S., Veeze H.J., and Tummler B. Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings. J. Clin. Invest. 108 (2001) 1705-1715
25. Brown A.J., Dusso A., and Slatopolsky E. Vitamin D. Am. J. Physiol. 277 (1999) F157-F175
26. Bryant S.H., and Conte-Camerino D. Chloride channel regulation in the skeletal muscle of normal and myotonic goats. Pflügers Arch. 417 (1991) 605-610
27. 2+ pump SERCA2b. Biochem. J. 330 (1998) 1015-1021
28. Carr G., Simmons N., and Sayer J. A role for CBS domain 2 in trafficking of chloride channel CLC-5. Biochem. Biophys. Res. Commun. 310 (2003) 600-605
29. Catalán M., Niemeyer M.I., Cid L.P., and Sepúlveda F.V. Basolateral ClC-2 chloride channels in surface colon epithelium: Regulation by a direct effect of intracellular chloride. Gastroenterology 126 (2004) 1104-1114
30. Cebotaru V., Kaul S., Devuyst O., Cai H., Racusen L., Guggino W.B., and Guggino S.E. High citrate diet delays progression of renal insufficiency in the ClC-5 knockout mouse model of Dent's disease. Kidney Int. 68 (2005) 642-652
31. Chalhoub N., Benachenhou N., Rajapurohitam V., Pata M., Ferron M., Frattini A., Villa A., and Vacher J. Grey-lethal mutation induces severe malignant autosomal recessive osteopetrosis in mouse and human. Nat. Med. 9 (2003) 399-406
32. Christensen E.I., Devuyst O., Dom G., Nielsen R., Van der Smissen P., Verroust P., Leruth M., Guggino W.B., and Courtoy P.J. 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 (2003) 8472-8477
33. Cid L.P., Montrose-Rafizadeh C., Smith D.I., Guggino W.B., and Cutting G.R. Cloning of a putative human voltage-gated chloride channel (CIC-2) cDNA widely expressed in human tissues. Hum. Mol. Genet. 4 (1995) 407-413
34. Cid L.P., Niemeyer M.I., Ramírez A., and Sepúlveda F.V. Splice variants of a ClC-2 chloride channel with differing functional characteristics. Am. J. Physiol. Cell Physiol. 279 (2000) C1198-C1210
35. Clarke L.L., Grubb B.R., Gabriel S.E., Smithies O., Koller B.H., and Boucher R.C. Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis. Science 257 (1992) 1125-1128
36. Cleiren E., Benichou O., Van Hul E., Gram J., Bollerslev J., Singer F.R., Beaverson K., Aledo A., Whyte M.P., Yoneyama T., deVernejoul M.C., and Van Hul W. Albers-Schönberg disease (autosomal dominant osteopetrosis, type II) results from mutations in the ClCN7 chloride channel gene. Hum. Mol. Genet. 10 (2001) 2861-2867
37. Cupers P., Veithen A., Hoekstra D., Baudhuin P., and Courtoy P.J. Three unrelated perturbations similarly uncouple fluid, bulk-membrane, and receptor endosomal flow in rat fetal fibroblasts. Biochem. Biophys. Res. Commun. 236 (1997) 661-664
38. D'Agostino D., Bertelli M., Gallo S., Cecchin S., Albiero E., Garofalo P.G., Gambardella A., St Hilaire J.M., Kwiecinski H., Andermann E., and Pandolfo M. Mutations and polymorphisms of the CLCN2 gene in idiopathic epilepsy. Neurology 63 (2004) 1500-1502
39. Davis-Kaplan S.R., Askwith C.C., Bengtzen A.C., Radisky D., and Kaplan J. 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 (1998) 13641-13645
40. De Angeli A., Monachello D., Ephritikhine G., Frachisse J.M., Thomine S., Gambale F., and Barbier-Brygoo H. The nitrate/proton antiporter AtCLCa mediates nitrate accumulation in plant vacuoles. Nature 442 (2006) 939-942
41. DeFazio R.A., Keros S., Quick M.W., and Hablitz J.J. Potassium-coupled chloride cotransport controls intracellular chloride in rat neocortical pyramidal neurons. J. Neurosci. 20 (2000) 8069-8076
42. Dent C.E., and Friedman M. Hypercalcuric rickets associated with renal tubular damage. Arch. Dis. Child. 39 (1964) 240-249
43. Devuyst O., Christie P.T., Courtoy P.J., Beauwens R., and Thakker R.V. Intra-renal and subcellular distribution of the human chloride channel, CLC-5, reveals a pathophysiological basis for Dent's disease. Hum. Mol. Genet. 8 (1999) 247-257
44. Dickerson L.W., Bonthius D.J., Schutte B.C., Yang B., Barna T.J., Bailey M.C., Nehrke K., Williamson R.A., and Lamb F.S. Altered GABAergic function accompanies hippocampal degeneration in mice lacking ClC-3 voltage-gated chloride channels. Brain Res. 958 (2002) 227-250
45. Duan D., Winter C., Cowley S., Hume J.R., and Horowitz B. Molecular identification of a volume-regulated chloride channel. Nature 390 (1997) 417-421
46. Dutzler R., Campbell E.B., Cadene M., Chait B.T., and MacKinnon R. X-ray structure of a ClC chloride channel at 3.0 Å reveals the molecular basis of anion selectivity. Nature 415 (2002) 287-294
47. Eggermont J., Buyse G., Voets T., Tytgat J., De Smedt H., Droogmans G., and Nilius B. Alternative splicing of ClC-6 (a member of the CIC chloride-channel family) transcripts generates three truncated isoforms one of which, ClC-6c, is kidney-specific. Biochem. J. 325 (1997) 269-276
48. +-secretion. Nature 414 (2001) 558-561
49. Facci L., Leon A., and Skaper S.D. Excitatory amino acid neurotoxicity in cultured retinal neurons: Involvement of N-methyl-D-aspartate (NMDA) and non-NMDA receptors and effect of ganglioside GM1. J. Neurosci. Res. 27 (1990) 202-210
50. Faundez V., and Hartzell H.C. Intracellular chloride channels: Determinants of function in the endosomal pathway. Sci. STKE 2004 233 (2004) re8
51. Ferroni S., Marchini C., Nobile M., and Rapisarda C. Characterization of an inwardly rectifying chloride conductance expressed by cultured rat cortical astrocytes. Glia 21 (1997) 217-227
52. Fisher S.E., Black G.C., Lloyd S.E., Hatchwell E., Wrong O., Thakker R.V., and Craig I.W. 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 (1994) 2053-2059
53. Fisher S.E., van Bakel I., Lloyd S.E., Pearce S.H., Thakker R.V., and Craig I.W. Cloning and characterization of CLCN5, the human kidney chloride channel gene implicated in Dent disease (an X-linked hereditary nephrolithiasis). Genomics 29 (1995) 598-606
54. - conductance cause hyperexcitability in recessive generalized myotonia (Becker). Muscle Nerve 14 (1991) 762-770
55. Frattini A., Orchard P.J., Sobacchi C., Giliani S., Abinun M., Mattsson J.P., Keeling D.J., Andersson A.K., Wallbrandt P., Zecca L., Notarangelo L.D., Vezzoni P., et al. Defects in TCIRG1 subunit of the vacuolar proton pump are responsible for a subset of human autosomal recessive osteopetrosis. Nat. Genet. 25 (2000) 343-346
56. Friedrich T., Breiderhoff T., and Jentsch T.J. Mutational analysis demonstrates that ClC-4 and ClC-5 directly mediate plasma membrane currents. J. Biol. Chem. 274 (1999) 896-902
57. Furukawa T., Horikawa S., Terai T., Ogura T., Katayama Y., and Hiraoka M. Molecular cloning and characterization of a novel truncated from (ClC-2 beta) of ClC-2 alpha (ClC-2G) in rabbit heart. [published erratum appears in FEBS Lett. February 10, 1997; 403(1), 111]. FEBS Lett. 375 (1995) 56-62
58. Furukawa T., Ogura T., Katayama Y., and Hiraoka M. Characteristics of rabbit ClC-2 current expressed in Xenopus oocytes and its contribution to volume regulation. Am. J. Physiol. 274 (1998) C500-C512
59. Gaxiola R.A., Yuan D.S., Klausner R.D., and Fink G.R. The yeast CLC chloride channel functions in cation homeostasis. Proc. Natl. Acad. Sci. USA 95 (1998) 4046-4050
60. Gaxiola R.A., Rao R., Sherman A., Grisafi P., Alper S.L., and Fink G.R. The Arabidopsis thaliana proton transporters, AtNhx1 and Avp1, can function in cation detoxification in yeast. Proc. Natl. Acad. Sci. USA 96 (1999) 1480-1485
61. Geelen D., Lurin C., Bouchez D., Frachisse J.M., Lelievre F., Courtial B., Barbier-Brygoo H., and Maurel C. Disruption of putative anion channel gene AtCLC-a in Arabidopsis suggests a role in the regulation of nitrate content. Plant J. 21 (2000) 259-267
62. Gentzsch M., Cui L., Mengos A., Chang X.B., Chen J.H., and Riordan J.R. The PDZ-binding chloride channel ClC-3B localizes to the Golgi and associates with CFTR-interacting PDZ proteins. J. Biol. Chem. 278 (2003) 6440-6449
63. George Jr. A.L., Bianchi L., Link E.M., and Vanoye C.G. From stones to bones: The biology of ClC chloride channels. Curr. Biol. 11 (2001) R620-R628
64. Greene J.R., Brown N.H., DiDomenico B.J., Kaplan J., and Eide D.J. 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 (1993) 542-553
65. Gronemeier M., Condie A., Prosser J., Steinmeyer K., Jentsch T.J., and Jockusch H. Nonsense and missense mutations in the muscular chloride channel gene Clc-1 of myotonic mice. J. Biol. Chem. 269 (1994) 5963-5967
66. Gründer S., Thiemann A., Pusch M., and Jentsch T.J. Regions involved in the opening of ClC-2 chloride channel by voltage and cell volume. Nature 360 (1992) 759-762
67. Gu F., and Gruenberg J. ARF1 regulates pH-dependent COP functions in the early endocytic pathway. J. Biol. Chem. 275 (2000) 8154-8160
68. Gulacsi A., Lee C.R., Sik A., Viitanen T., Kaila K., Tepper J.M., and Freund T.F. Cell type-specific differences in chloride-regulatory mechanisms and GABA(A) receptor-mediated inhibition in rat substantia nigra. J. Neurosci. 23 (2003) 8237-8246
69. Günther W., Lüchow A., Cluzeaud F., Vandewalle A., and Jentsch T.J. 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 (1998) 8075-8080
70. Günther W., Piwon N., and Jentsch T.J. The ClC-5 chloride channel knock-out mouse-an animal model for Dent's disease. Pflügers Arch. 445 (2003) 456-462
71. Gyömörey K., Yeger H., Ackerley C., Garami E., and Bear C.E. Expression of the chloride channel ClC-2 in the murine small intestine epithelium. Am. J. Physiol. Cell Physiol. 279 (2000) C1787-C1794
72. Hara-Chikuma M., Wang Y., Guggino S.E., Guggino W.B., and Verkman A.S. Impaired acidification in early endosomes of ClC-5 deficient proximal tubule. Biochem. Biophys. Res. Commun. 329 (2005) 941-946
73. Hara-Chikuma M., Yang B., Sonawane N.D., Sasaki S., Uchida S., and Verkman A.S. ClC-3 chloride channels facilitate endosomal acidification and chloride accumulation. J. Biol. Chem. 280 (2005) 1241-1247
74. Haug K., Warnstedt M., Alekov A.K., Sander T., Ramirez A., Poser B., Maljevic S., Hebeisen S., Kubisch C., Rebstock J., Horvath S., Hallmann K., et al. Mutations in CLCN2 encoding a voltage-gated chloride channel are associated with idiopathic generalized epilepsies. Nat. Genet. 33 (2003) 527-532
75. Hechenberger M., Schwappach B., Fischer W.N., Frommer W.B., Jentsch T.J., and Steinmeyer K. A family of putative chloride channels from Arabidopsis and functional complementation of a yeast strain with a CLC gene disruption. J. Biol. Chem. 271 (1996) 33632-33638
76. Holroyd C., Kistner U., Annaert W., and Jahn R. Fusion of endosomes involved in synaptic vesicle recycling. Mol. Biol. Cell 10 (1999) 3035-3044
77. Hoopes Jr. R.R., Raja K.M., Koich A., Hueber P., Reid R., Knohl S.J., and Scheinman S.J. Evidence for genetic heterogeneity in Dent's disease. Kidney Int. 65 (2004) 1615-1620
78. Hoopes Jr. R.R., Shrimpton A.E., Knohl S.J., Hueber P., Hoppe B., Matyus J., Simckes A., Tasic V., Toenshoff B., Suchy S.F., Nussbaum R.L., and Scheinman S.J. Dent disease with mutations in OCRL1. Am. J. Hum. Genet. 76 (2005) 260-267
79. Hryciw D.H., Ekberg J., Lee A., Lensink I.L., Kumar S., Guggino W.B., Cook D.I., Pollock C.A., and Poronnik P. Nedd4-2 functionally interacts with ClC-5: Involvement in constitutive albumin endocytosis in proximal tubule cells. J. Biol. Chem. 279 (2004) 54996-55007
80. Hübner C., Stein V., Hermanns-Borgmeyer I., Meyer T., Ballanyi K., and Jentsch T.J. Disruption of KCC2 reveals an essential role of K-Cl-cotransport already in early synaptic inhibition. Neuron 30 (2001) 515-524
81. Iaizzo P.A., Franke C., Hatt H., Spittelmeister W., Ricker K., Rudel R., and Lehmann-Horn F. Altered sodium channel behaviour causes myotonia in dominantly inherited myotonia congenita. Neuromuscul. Disord. 1 (1991) 47-53
82. Igarashi T., Günther W., Sekine T., Inatomi J., Shiraga H., Takahashi S., Suzuki J., Tsuru N., Yanagihara T., Shimazu M., Jentsch T.J., and Thakker R.V. Functional characterization of renal chloride channel, CLCN5, mutations associated with Dent's Japan disease. Kidney Int. 54 (1998) 1850-1856
83. Isnard-Bagnis C., Da Silva N., Beaulieu V., Yu A.S., Brown D., and Breton S. Detection of ClC-3 and ClC-5 in epididymal epithelium: Immunofluorescence and RT-PCR after LCM. Am. J. Physiol. Cell Physiol. 284 (2003) C220-C232
84. Jentsch T.J., Steinmeyer K., and Schwarz G. Primary structure of Torpedo marmorata chloride channel isolated by expression cloning in Xenopus oocytes. Nature 348 (1990) 510-514
85. Jentsch T.J., Günther W., Pusch M., and Schwappach B. Properties of voltage-gated chloride channels of the ClC gene family. J. Physiol. (Lond.) 482 (1995) 19S-25S
86. Jentsch T.J., Stein V., Weinreich F., and Zdebik A.A. Molecular structure and physiological function of chloride channels. Physiol. Rev. 82 (2002) 503-568
87. Johnson Jr. R.G. Proton pumps and chemiosmotic coupling as a generalized mechanism for neurotransmitter and hormone transport. Ann. N. Y. Acad. Sci. 493 (1987) 162-177
88. Jordt S.E., and Jentsch T.J. Molecular dissection of gating in the ClC-2 chloride channel. EMBO J. 16 (1997) 1582-1592
89. Kaila K., Lamsa K., Smirnov S., Taira T., and Voipio J. Long-lasting GABA-mediated depolarization evoked by high-frequency stimulation in pyramidal neurons of rat hippocampal slice is attributable to a network-driven, bicarbonate-dependent K+ transient. J. Neurosci. 17 (1997) 7662-7672
90. Kasper D., Planells-Cases R., Fuhrmann J.C., Scheel O., Zeitz O., Ruether K., Schmitt A., Poet M., Steinfeld R., Schweizer M., Kornak U., and Jentsch T.J. Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration. EMBO J. 24 (2005) 1079-1091
91. Kawasaki M., Uchida S., Monkawa T., Miyawaki A., Mikoshiba K., Marumo F., and Sasaki S. Cloning and expression of a protein kinase C-regulated chloride channel abundantly expressed in rat brain neuronal cells. Neuron 12 (1994) 597-604
92. Kawasaki M., Suzuki M., Uchida S., Sasaki S., and Marumo F. Stable and functional expression of the ClC-3 chloride channel in somatic cell lines. Neuron 14 (1995) 1285-1291
93. Kida Y., Uchida S., Miyazaki H., Sasaki S., and Marumo F. Localization of mouse CLC-6 and CLC-7 mRNA and their functional complementation of yeast CLC gene mutant. Histochem. Cell Biol. 115 (2001) 189-194
94. Kieferle S., Fong P., Bens M., Vandewalle A., and Jentsch T.J. Two highly homologous members of the ClC chloride channel family in both rat and human kidney. Proc. Natl. Acad. Sci. USA 91 (1994) 6943-6947
95. Koch M.C., Steinmeyer K., Lorenz C., Ricker K., Wolf F., Otto M., Zoll B., Lehmann-Horn F., Grzeschik K.H., and Jentsch T.J. The skeletal muscle chloride channel in dominant and recessive human myotonia. Science 257 (1992) 797-800
96. +-ATPase cause infantile malignant osteopetrosis. Hum. Mol. Genet. 9 (2000) 2059-2063
97. Kornak U., Kasper D., Bösl M.R., Kaiser E., Schweizer M., Schulz A., Friedrich W., Delling G., and Jentsch T.J. Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man. Cell 104 (2001) 205-215
98. Kubisch C., Schmidt-Rose T., Fontaine B., Bretag A.H., and Jentsch T.J. ClC-1 chloride channel mutations in myotonia congenita: Variable penetrance of mutations shifting the voltage dependence. Hum. Mol. Genet. 7 (1998) 1753-1760
99. Lange P.F., Wartosch L., Jentsch T.J., and Fuhrmann J.C. ClC-7 requires Ostm1 as a β-subunit to support bone resorption and lysosomal function. Nature 440 (2006) 220-223
100. Leheste J.R., Rolinski B., Vorum H., Hilpert J., Nykjaer A., Jacobsen C., Aucouturier P., Moskaug J.O., Otto A., Christensen E.I., and Willnow T.E. Megalin knockout mice as an animal model of low molecular weight proteinuria. Am. J. Pathol. 155 (1999) 1361-1370
101. Li X., Shimada K., Showalter L.A., and Weinman S.A. Biophysical properties of ClC-3 differentiate it from swelling-activated chloride channels in Chinese hamster ovary-K1 cells. J. Biol. Chem. 275 (2000) 35994-35998
102. Li X., Wang T., Zhao Z., and Weinman S.A. The ClC-3 chloride channel promotes acidification of lysosomes in CHO-K1 and Huh-7 cells. Am. J. Physiol. Cell Physiol. 282 (2002) C1483-C1491
103. Li Y., Kane T., Tipper C., Spatrick P., and Jenness D.D. Yeast mutants affecting possible quality control of plasma membrane proteins. Mol. Cell. Biol. 19 (1999) 3588-3599
104. Lipecka J., Bali M., Thomas A., Fanen P., Edelman A., and Fritsch J. Distribution of ClC-2 chloride channel in rat and human epithelial tissues. Am. J. Physiol. Cell Physiol. 282 (2002) C805-C816
105. Lipicky R.J., and Bryant S.H. Sodium, potassium, and chloride fluxes in intercostal muscle from normal goats and goats with hereditary myotonia. J. Gen. Physiol. 50 (1966) 89-111
106. Lipicky R.J., Bryant S.H., and Salmon J.H. 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 (1971) 2091-2103
107. Lloyd S.E., Pearce S.H., Fisher S.E., Steinmeyer K., Schwappach B., Scheinman S.J., Harding B., Bolino A., Devoto M., Goodyer P., Rigden S.P., Wrong O., et al. A common molecular basis for three inherited kidney stone diseases. Nature 379 (1996) 445-449
108. Lloyd S.E., Günther W., Pearce S.H., Thomson A., Bianchi M.L., Bosio M., Craig I.W., Fisher S.E., Scheinman S.J., Wrong O., Jentsch T.J., and Thakker R.V. Characterisation of renal chloride channel, CLCN5, mutations in hypercalciuric nephrolithiasis (kidney stones) disorders. Hum. Mol. Genet. 6 (1997) 1233-1239
109. Lorenz C., Pusch M., and Jentsch T.J. Heteromultimeric CLC chloride channels with novel properties. Proc. Natl. Acad. Sci. USA 93 (1996) 13362-13366
110. Ludwig M., and Utsch B. Dent disease-like phenotype and the chloride channel ClC-4 (CLCN4) gene. Am. J. Med. Genet. A 128 (2004) 434-435
111. Ludwig M., Waldegger S., Nuutinen M., Bokenkamp A., Reissinger A., Steckelbroeck S., and Utsch B. 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 (2003) 176-184
112. Lurin C., Geelen D., Barbier-Brygoo H., Guern J., and Maurel C. Cloning and functional expression of a plant voltage-dependent chloride channel. Plant Cell 8 (1996) 701-711
113. Lurin C., Güclü J., Cheniclet C., Carde J.P., Barbier-Brygoo H., and Maurel C. CLC-Nt1, a putative chloride channel protein of tobacco, co-localizes with mitochondrial membrane markers. Biochem. J. 348 (2000) 291-295
114. Makara J.K., Petheo G.L., Toth A., and Spat A. pH-sensitive inwardly rectifying chloride current in cultured rat cortical astrocytes. Glia 34 (2001) 52-58
115. - currents regulated during development and after injury. Mol. Cell. Neurosci. 23 (2003) 521-530
116. - channel. Am. J. Physiol. 268 (1995) C191-C200
117. Maranda B., Brown D., Bourgoin S., Casanova J.E., Vinay P., Ausiello D.A., and Marshansky V. Intra-endosomal pH-sensitive recruitment of the Arf-nucleotide exchange factor ARNO and Arf6 from cytoplasm to proximal tubule endosomes. J. Biol. Chem. 276 (2001) 18540-18550
118. Marini C., Scheffer I.E., Crossland K.M., Grinton B.E., Phillips F.L., McMahon J.M., Turner S.J., Dean J.T., Kivity S., Mazarib A., Neufeld M.Y., Korczyn A.D., et al. Genetic architecture of idiopathic generalized epilepsy: Clinical genetic analysis of 55 multiplex families. Epilepsia 45 (2004) 467-478
119. 3 target genes in ClC-5 KO mice. Kidney Int. 70 (2006) 79-87
120. Marshansky V., Ausiello D.A., and Brown D. Physiological importance of endosomal acidification: Potential role in proximal tubulopathies. Curr. Opin. Nephrol. Hypertens. 11 (2002) 527-537
121. Matsumura Y., Uchida S., Kondo Y., Miyazaki H., Ko S.B., Hayama A., Morimoto T., Liu W., Arisawa M., Sasaki S., and Marumo F. Overt nephrogenic diabetes insipidus in mice lacking the CLC-K1 chloride channel. Nat. Genet. 21 (1999) 95-98
122. Maycox P.R., Deckwerth T., Hell J.W., and Jahn R. Glutamate uptake by brain synaptic vesicles. Energy dependence of transport and functional reconstitution in proteoliposomes. J. Biol. Chem. 263 (1988) 15423-15428
123. Metz J., Wachter A., Schmidt B., Bujnicki J.M., and Schwappach B. The yeast Arr4p ATPase binds the chloride transporter Gef1p when copper is available in the cytosol. J. Biol. Chem. 281 (2006) 410-417
124. Meyer S., and Dutzler R. Crystal Structure of the cytoplasmic domain of the chloride channel ClC-0. Structure 14 (2006) 299-307
125. Meyer-Kleine C., Steinmeyer K., Ricker K., Jentsch T.J., and Koch M.C. Spectrum of mutations in the major human skeletal muscle chloride channel gene (CLCN1) leading to myotonia. Am. J. Hum. Genet. 57 (1995) 1325-1334
126. Miles R. Neurobiology. A homeostatic switch. Nature 397 (1999) 215-216
127. Miller C., and White M.M. Dimeric structure of single chloride channels from Torpedo electroplax. Proc. Natl. Acad. Sci. USA 81 (1984) 2772-2775
128. Mohammad-Panah R., Gyomorey K., Rommens J., Choudhury M., Li C., Wang Y., and Bear C.E. ClC-2 contributes to native chloride secretion by a human intestinal cell line, Caco-2. J. Biol. Chem. 276 (2001) 8306-8313
129. Mohammad-Panah R., Harrison R., Dhani S., Ackerley C., Huan L.J., Wang Y., and Bear C.E. The chloride channel ClC-4 contributes to endosomal acidification and trafficking. J. Biol. Chem. 278 (2003) 29267-29277
130. Morimoto T., Uchida S., Sakamoto H., Kondo Y., Hanamizu H., Fukui M., Tomino Y., Nagano N., Sasaki S., and Marumo F. Mutations in CLCN5 chloride channel in Japanese patients with low molecular weight proteinuria. J. Am. Soc. Nephrol. 9 (1998) 811-818
131. Murray C.B., Morales M.M., Flotte T.R., McGrath-Morrow S.A., Guggino W.B., and Zeitlin P.L. CLC-2: A developmentally dependent chloride channel expressed in the fetal lung and downregulated after birth. Am. J. Respir. Cell Mol. Biol. 12 (1995) 597-604
132. Murray C.B., Chu S., and Zeitlin P.L. Gestational and tissue-specific regulation of ClC-2 chloride channel expression. Am. J. Physiol. 271 (1996) L829-L837
133. Nehrke K., Begenisich T., Pilato J., and Melvin J.E. Into ion channel and transporter function. Caenorhabditis elegans ClC-type chloride channels: Novel variants and functional expression. Am. J. Physiol. Cell Physiol. 279 (2000) C2052-C2066
134. - current in salivary acinar cells from Clcn2 knockout mice. J. Biol. Chem. 26 (2002) 23604-23611
135. Niemeyer M.I., Cid L.P., Zuniga L., Catalán M., and Sepúlveda F.V. A conserved pore-lining glutamate as a voltage- and chloride-dependent gate in the ClC-2 chloride channel. J. Physiol. 553 (2003) 873-879
136. Niemeyer M.I., Yusef Y.R., Cornejo I., Flores C.A., Sepúlveda F.V., and Cid L.P. Functional evaluation of human ClC-2 chloride channel mutations associated with idiopathic generalized epilepsies. Physiol. Genomics 19 (2004) 74-83
137. Nobile M., Pusch M., Rapisarda C., and Ferroni S. Single-channel analysis of a ClC-2-like chloride conductance in cultured rat cortical astrocytes. FEBS Lett. 479 (2000) 10-14
138. Norden A.G., Lapsley M., Lee P.J., Pusey C.D., Scheinman S.J., Tam F.W., Thakker R.V., Unwin R.J., and Wrong O. Glomerular protein sieving and implications for renal failure in Fanconi syndrome. Kidney Int. 60 (2001) 1885-1892
139. Obermüller N., Gretz N., Kriz W., Reilly R.F., and Witzgall R. 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 epithelial cells. J. Clin. Invest. 101 (1998) 635-642
140. Ogura T., Furukawa T., Toyozaki T., Yamada K., Zheng Y.J., Katayama Y., Nakaya H., and Inagaki N. ClC-3B, a novel ClC-3 splicing variant that interacts with EBP50 and facilitates expression of CFTR-regulated ORCC. FASEB J. 16 (2002) S63-S65
141. - channels in rat parotid acinar cells with ClC-2 in a mammalian expression system. J. Membr. Biol. 163 (1998) 87-95
142. Parsons S.M., Prior C., and Marshall I.G. Acetylcholine transport, storage, and release. Int. Rev. Neurobiol. 35 (1993) 279-390
143. Pena-Munzenmayer G., Catalan M., Cornejo I., Figueroa C.D., Melvin J.E., Niemeyer M.I., Cid L.P., and Sepulveda F.V. Basolateral localization of native ClC-2 chloride channels in absorptive intestinal epithelial cells and basolateral sorting encoded by a CBS-2 domain di-leucine motif. J. Cell Sci. 118 (2005) 4243-4252
144. Petalcorin M.I., Oka T., Koga M., Ogura K., Wada Y., Ohshima Y., and Futai M. Disruption of clh-1, a chloride channel gene, results in a wider body of Caenorhabditis elegans. J. Mol. Biol. 294 (1999) 347-355
145. Pfeiffer F., Graham D., and Betz H. Purification by affinity chromatography of the glycine receptor of rat spinal cord. J. Biol. Chem. 257 (1982) 9389-9393
146. Picollo A., and Pusch M. Chloride/proton antiporter activity of mammalian CLC proteins ClC-4 and ClC-5. Nature 436 (2005) 420-423
147. Pirozzi G., McConnell S.J., Uveges A.J., Carter J.M., Sparks A.B., Kay B.K., and Fowlkes D.M. Identification of novel human WW domain-containing proteins by cloning of ligand targets. J. Biol. Chem. 272 (1997) 14611-14616
148. --channel disruption impairs endocytosis in a mouse model for Dent's disease. Nature 408 (2000) 369-373
149. Poët M., Kornak U., Schweizer M., Zdebik A.A., Scheel O., Hoelter S., Wurst W., Schmitt A., Fuhrmann J.C., Planells-Cases R., Mole S.E., Hübner C.A., and Jentsch T.J. Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6. Proc. Natl. Acad. Sci. USA 103 (2000) 13854-13859
150. Pusch M. Myotonia caused by mutations in the muscle chloride channel gene CLCN1. Hum. Mutat. 19 (2002) 423-434
151. Pusch M., Ludewig U., Rehfeldt A., and Jentsch T.J. Gating of the voltage-dependent chloride channel ClC-0 by the permeant anion. Nature 373 (1995) 527-531
152. Pusch M., Steinmeyer K., Koch M.C., and Jentsch T.J. Mutations in dominant human myotonia congenita drastically alter the voltage dependence of the ClC-1 chloride channel. Neuron 15 (1995) 1455-1463
153. Pusch M., Jordt S.E., Stein V., and Jentsch T.J. Chloride dependence of hyperpolarization-activated chloride channel gates. J. Physiol. (Lond.) 515 (1999) 341-353
154. Riordan J.R., Rommens J.M., Kerem B., Alon N., Rozmahel R., Grzelczak Z., Zielenski J., Lok S., Plavsic N., Chou J.L., Drumm M.L., Iannuzzi M.C., et al. Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. Science 245 (1989) 1066-1073
155. - co-transporter KCC2 renders GABA hyperpolarizing during neuronal maturation. Nature 397 (1999) 251-255
156. Rotin D., Staub O., and Haguenauer-Tsapis R. Ubiquitination and endocytosis of plasma membrane proteins: Role of Nedd4/Rsp5p family of ubiquitin-protein ligases. J. Membr. Biol. 176 (2000) 1-17
157. Rozmahel R., Wilschanski M., Matin A., Plyte S., Oliver M., Auerbach W., Moore A., Forstner J., Durie P., Nadeau J., Bear C., and Tsui L.C. Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor. Nat. Genet. 12 (1996) 280-287
158. Rüdel R., and Lehmann-Horn F. Membrane changes in cells from myotonia patients. Physiol. Rev. 65 (1985) 310-356
159. Rugarli E.I., Adler D.A., Borsani G., Tsuchiya K., Franco B., Hauge X., Disteche C., Chapman V., and Ballabio A. Different chromosomal localization of the Clcn4 gene in Mus spretus and C57BL/6J mice. Nat. Genet. 10 (1995) 466-471
160. Rutledge E., Bianchi L., Christensen M., Boehmer C., Morrison R., Broslat A., Beld A.M., George A.L., Greenstein D., and Strange K. CLH-3, a ClC-2 anion channel ortholog activated during meiotic maturation in C. elegans oocytes. Curr. Biol. 11 (2001) 161-170
161. Sabolic I., and Burckhardt G. Characteristics of the proton pump in rat renal cortical endocytotic vesicles. Am. J. Physiol. 250 (1986) F817-F826
162. +-ATPase. Am. J. Physiol. 277 (1999) F957-F965
163. Salazar G., Love R., Styers M.L., Werner E., Peden A., Rodriguez S., Gearing M., Wainer B.H., and Faundez V. AP-3-dependent mechanisms control the targeting of a chloride channel (ClC-3) in neuronal and non-neuronal cells. J. Biol. Chem. 279 (2004) 25430-25439
164. Salvatore F., Scudiero O., and Castaldo G. Genotype-phenotype correlation in cystic fibrosis: The role of modifier genes. Am. J. Med. Genet. 111 (2002) 88-95
165. Sander T., Schulz H., Saar K., Gennaro E., Riggio M.C., Bianchi A., Zara F., Luna D., Bulteau C., Kaminska A., Ville D., Cieuta C., et al. Genome search for susceptibility loci of common idiopathic generalised epilepsies. Hum. Mol. Genet. 9 (2000) 1465-1472
166. Scheel O., Zdebik A., Lourdel S., and Jentsch T.J. Voltage-dependent electrogenic chloride proton exchange by endosomal CLC proteins. Nature 436 (2005) 424-427
167. Scheinman S.J. X-linked hypercalciuric nephrolithiasis: Clinical syndromes and chloride channel mutations. Kidney Int. 53 (1998) 3-17
168. Schlingmann K.P., Konrad M., Jeck N., Waldegger P., Reinalter S.C., Holder M., Seyberth H.W., and Waldegger S. Salt wasting and deafness resulting from mutations in two chloride channels. N. Engl. J. Med. 350 (2004) 1314-1319
169. Schofield P.R., Darlison M.G., Fujita N., Burt D.R., Stephenson F.A., Rodriguez H., Rhee L.M., Ramachandran J., Reale V., Glencorse T.A., Seeburg P.H., and Barnard E.A. Sequence and functional expression of the GABA A receptor shows a ligand-gated receptor super-family. Nature 328 (1987) 221-227
170. Schriever A.M., Friedrich T., Pusch M., and Jentsch T.J. CLC chloride channels in Caenorhabditis elegans. J. Biol. Chem. 274 (1999) 34238-34244
171. --channel mutated in Dent's disease. J. Biol. Chem. 276 (2001) 12049-12054
172. 2 and COOH terminus of the yeast CLC putative chloride channel Gef1p. J. Biol. Chem. 273 (1998) 15110-15118
173. Schwiebert E.M., Cid-Soto L.P., Stafford D., Carter M., Blaisdell C.J., Zeitlin P.L., Guggino W.B., and Cutting G.R. Analysis of ClC-2 channels as an alternative pathway for chloride conduction in cystic fibrosis airway cells. Proc. Natl. Acad. Sci. USA 95 (1998) 3879-3884
174. Scimeca J.C., Franchi A., Trojani C., Parrinello H., Grosgeorge J., Robert C., Jaillon O., Poirier C., Gaudray P., and Carle G.F. The gene encoding the mouse homologue of the human osteoclast-specific 116-kDa V-ATPase subunit bears a deletion in osteosclerotic (oc/oc) mutants. Bone 26 (2000) 207-213
175. Scott J.W., Hawley S.A., Green K.A., Anis M., Stewart G., Scullion G.A., Norman D.G., and Hardie D.G. CBS domains form energy-sensing modules whose binding of adenosine ligands is disrupted by disease mutations. J. Clin. Invest. 113 (2004) 274-284
176. Seong E., Wainer B.H., Hughes E.D., Saunders T.L., Burmeister M., and Faundez V. Genetic analysis of the neuronal and ubiquitous AP-3 adaptor complexes reveals divergent functions in brain. Mol. Biol. Cell 16 (2005) 128-140
177. Sik A., Smith R.L., and Freund T.F. Distribution of chloride channel-2-immunoreactive neuronal and astrocytic processes in the hippocampus. Neuroscience 101 (2000) 51-65
178. Silva I.V., Cebotaru V., Wang H., Wang X.T., Wang S.S., Guo G., Devuyst O., Thakker R.V., Guggino W.B., and Guggino S.E. The ClC-5 knockout mouse model of Dent's disease has renal hypercalciuria and increased bone turnover. J. Bone Miner. Res. 18 (2003) 615-623
179. Simon D.B., Karet F.E., Hamdan J.M., DiPietro A., Sanjad S.A., and Lifton R.P. Bartter's syndrome, hypokalaemic alkalosis with hypercalciuria, is caused by mutations in the Na-K-2Cl cotransporter NKCC2. Nat. Genet. 13 (1996) 183-188
180. + channel, ROMK. Nat. Genet. 14 (1996) 152-156
181. Simon D.B., Bindra R.S., Mansfield T.A., Nelson-Williams C., Mendonca E., Stone R., Schurman S., Nayir A., Alpay H., Bakkaloglu A., Rodriguez-Soriano J., Morales J.M., et al. Mutations in the chloride channel gene, CLCNKB, cause Bartter's syndrome type III. Nat. Genet. 17 (1997) 171-178
182. Smith R.L., Clayton G.H., Wilcox C.L., Escudero K.W., and Staley K.J. 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 (1995) 4057-4067
183. Staley K. The role of an inwardly rectifying chloride conductance in postsynaptic inhibition. J. Neurophysiol. 72 (1994) 273-284
184. A receptor function following gene transfer of the CLC-2 chloride channel. Neuron 17 (1996) 543-551
185. Steinmeyer K., Klocke R., Ortland C., Gronemeier M., Jockusch H., Gründer S., and Jentsch T.J. Inactivation of muscle chloride channel by transposon insertion in myotonic mice. Nature 354 (1991) 304-308
186. Steinmeyer K., Ortland C., and Jentsch T.J. Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel. Nature 354 (1991) 301-304
187. Steinmeyer K., Lorenz C., Pusch M., Koch M.C., and Jentsch T.J. Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen). EMBO J. 13 (1994) 737-743
188. Steinmeyer K., Schwappach B., Bens M., Vandewalle A., and Jentsch T.J. Cloning and functional expression of rat CLC-5, a chloride channel related to kidney disease. J. Biol. Chem. 270 (1995) 31172-31177
189. Stobrawa S.M., Breiderhoff T., Takamori S., Engel D., Schweizer M., Zdebik A.A., Bösl M.R., Ruether K., Jahn H., Draguhn A., Jahn R., and Jentsch T.J. Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus. Neuron 29 (2001) 185-196
190. Strong T.V., Boehm K., and Collins F.S. Localization of cystic fibrosis transmembrane conductance regulator mRNA in the human gastrointestinal tract by in situ hybridization. J. Clin. Invest. 93 (1994) 347-354
191. Südhof T.C. The synaptic vesicle cycle revisited. Neuron 28 (2000) 317-320
192. Suzuki T., Rai T., Hayama A., Sohara E., Suda S., Itoh T., Sasaki S., and Uchida S. Intracellular localization of ClC chloride channels and their ability to form hetero-oligomers. J. Cell. Physiol. 206 (2006) 792-798
193. Teardo E., Frare E., Segalla A., De Marco V., Giacometti G.M., and Szabo I. Localization of a putative ClC chloride channel in spinach chloroplasts. FEBS Lett. 579 (2005) 4991-4996
194. Thiemann A., Gründer S., Pusch M., and Jentsch T.J. A chloride channel widely expressed in epithelial and non-epithelial cells. Nature 356 (1992) 57-60
195. Thomsen J. Tonische Krämpfe in willkürlich beweglichen Muskeln in Folge von ererbter psychischer Disposition. Arch. Psychiatr. Nervenkr. 6 (1876) 702-718
196. Tyteca D., Van Der Smissen P., Mettlen M., Van Bambeke F., Tulkens P.M., Mingeot-Leclercq M.P., and Courtoy P.J. 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 (2002) 86-100
197. Uchida S., Sasaki S., Furukawa T., Hiraoka M., Imai T., Hirata Y., and Marumo F. Molecular cloning of a chloride channel that is regulated by dehydration and expressed predominantly in kidney medulla [published erratum appears in J. Biol. Chem. July 22, 1994, 269(29), 19192]. J. Biol. Chem. 268 (1993) 3821-3824
198. Uchida S., Sasaki S., Nitta K., Uchida K., Horita S., Nihei H., and Marumo F. Localization and functional characterization of rat kidney-specific chloride channel, ClC-K1. J. Clin. Invest. 95 (1995) 104-113
199. van Slegtenhorst M.A., Bassi M.T., Borsani G., Wapenaar M.C., Ferrero G.B., de Conciliis L., Rugarli E.I., Grillo A., Franco B., Zoghbi H.Y., and Ballabio A. A gene from the Xp22.3 region shares homology with voltage-gated chloride channels. Hum. Mol. Genet. 3 (1994) 547-552
200. Vandewalle A., Cluzeaud F., Bens M., Kieferle S., Steinmeyer K., and Jentsch T.J. Localization and induction by dehydration of ClC-K chloride channels in the rat kidney. Am. J. Physiol. 272 (1997) F678-F688
201. Vandewalle A., Cluzeaud F., Peng K.C., Bens M., Lüchow A., Günther W., and Jentsch T.J. Tissue distribution and subcellular localization of the ClC-5 chloride channel in rat intestinal cells. Am. J. Physiol. Cell Physiol. 280 (2001) C373-C381
202. Varela D., Niemeyer M.I., Cid L.P., and Sepulveda F.V. Effect of an N-terminus deletion on voltage-dependent gating of the ClC-2 chloride channel. J. Physiol. 544 (2002) 363-372
203. Wächter A., and Schwappach B. The yeast CLC chloride channel is proteolytically processed by the furin-like protease Kex2p in the first extracellular loop. FEBS Lett. 579 (2005) 1149-1153
204. Waldegger S., and Jentsch T.J. Functional and structural analysis of ClC-K chloride channels involved in renal disease. J. Biol. Chem. 275 (2000) 24527-24533
205. Walz W. Chloride/anion channels in glial cell membranes. Glia 40 (2002) 1-10
206. Wang S.S., Devuyst O., Courtoy P.J., Wang X.T., Wang H., Wang Y., Thakker R.V., Guggino S., and Guggino W.B. 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 (2000) 2937-2945
207. Wang T., and Weinman S.A. Involvement of chloride channels in hepatic copper metabolism: ClC-4 promotes copper incorporation into ceruloplasmin. Gastroenterology 126 (2004) 1157-1166
208. Weinreich F., and Jentsch T.J. Pores formed by single subunits in mixed dimers of different CLC chloride channels. J. Biol. Chem. 276 (2001) 2347-2353
209. Welsh M.J., Smith P.L., Fromm M., and Frizzell R.A. Crypts are the site of intestinal fluid and electrolyte secretion. Science 218 (1982) 1219-1221
210. Weylandt K.H., Valverde M.A., Nobles M., Raguz S., Amey J.S., Díaz M., Nastrucci C., Higgins C.F., and Sardini A. Human ClC-3 is not the swelling-activated chloride channel involved in cell volume regulation. J. Biol. Chem. 276 (2001) 17461-17467
211. White M.M., and Miller C. A voltage-gated anion channel from the electric organ of Torpedo californica. J. Biol. Chem. 254 (1979) 10161-10166
212. Wrong O.M., Norden A.G., and Feest T.G. 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 (1994) 473-493
213. Wu F., Roche P., Christie P.T., Loh N.Y., Reed A.A., Esnouf R.M., and Thakker R.V. Modeling study of human renal chloride channel (hCLC-5) mutations suggests a structural-functional relationship. Kidney Int. 63 (2003) 1426-1432
214. Xiong H., Li C., Garami E., Wang Y., Ramjeesingh M., Galley K., and Bear C.E. ClC-2 activation modulates regulatory volume decrease. J. Membr. Biol. 167 (1999) 215-221
215. -/- mice. J. Physiol. 557 (2004) 439-456
216. Yoshikawa M., Uchida S., Ezaki J., Rai T., Hayama A., Kobayashi K., Kida Y., Noda M., Koike M., Uchiyama Y., Marumo F., Kominami E., et al. CLC-3 deficiency leads to phenotypes similar to human neuronal ceroid lipofuscinosis. Genes Cells 7 (2002) 597-605
217. - channel does not exacerbate the cystic fibrosis phenotype of CFTR mouse models. J. Biol. Chem. 279 (2004) 22276-22283