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Physiological Reviews, Vol. 82, No. 2, April 2002, pp. 503-568; 10.1152/physrev.00029.2001.
Copyright ©2002 by the American Physiological Society
Zentrum für Molekulare Neurobiologie Hamburg, Universität Hamburg, Hamburg, Germany
Jentsch, Thomas J.,
Valentin Stein,
Frank Weinreich, and
Anselm A. Zdebik.
Molecular Structure and Physiological Function of Chloride
Channels. Physiol. Rev. 82: 503-568, 2002.
Cl
channels reside both in
the plasma membrane and in intracellular organelles. Their functions
range from ion homeostasis to cell volume regulation, transepithelial
transport, and regulation of electrical excitability. Their
physiological roles are impressively illustrated by various inherited
diseases and knock-out mouse models. Thus the loss of distinct
Cl channels leads to an impairment of transepithelial
transport in cystic fibrosis and Bartter's syndrome, to increased
muscle excitability in myotonia congenita, to reduced endosomal
acidification and impaired endocytosis in Dent's disease, and to
impaired extracellular acidification by osteoclasts and osteopetrosis.
The disruption of several Cl channels in mice results in
blindness. Several classes of Cl channels have not yet
been identified at the molecular level. Three molecularly distinct
Cl channel families (CLC, CFTR, and ligand-gated GABA
and glycine receptors) are well established. Mutagenesis and functional
studies have yielded considerable insights into their structure and
function. Recently, the detailed structure of bacterial CLC proteins
was determined by X-ray analysis of three-dimensional crystals.
Nonetheless, they are less well understood than cation channels and
show remarkably different biophysical and structural properties. Other
gene families (CLIC or CLCA) were also reported to encode
Cl channels but are less well characterized. This review
focuses on molecularly identified Cl channels and their
physiological roles.
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Y. R. Yusef, L. Zuniga, M. Catalan, M. I. Niemeyer, L. P. Cid, and F. V. Sepulveda Removal of gating in voltage-dependent ClC-2 chloride channel by point mutations affecting the pore and C-terminus CBS-2 domain J. Physiol., April 1, 2006; 572(1): 173 - 181. [Abstract] [Full Text] [PDF]
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J. Denton, K. Nehrke, X. Yin, A. M. Beld, and K. Strange Altered gating and regulation of a carboxy-terminal ClC channel mutant expressed in the Caenorhabditis elegans oocyte Am J Physiol Cell Physiol, April 1, 2006; 290(4): C1109 - C1118. [Abstract] [Full Text] [PDF]
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 M.-F. van den Hove, K. Croizet-Berger, F. Jouret, S. E. Guggino, W. B. Guggino, O. Devuyst, and P. J. Courtoy The Loss of the Chloride Channel, ClC-5, Delays Apical Iodide Efflux and Induces a Euthyroid Goiter in the Mouse Thyroid Gland Endocrinology, March 1, 2006; 147(3): 1287 - 1296. [Abstract] [Full Text] [PDF]
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K. W. Beyenbach and H. Wieczorek The V-type H+ ATPase: molecular structure and function, physiological roles and regulation J. Exp. Biol., February 15, 2006; 209(4): 577 - 589. [Abstract] [Full Text] [PDF]
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J. Metz, A. Wachter, B. Schmidt, J. M. Bujnicki, and B. Schwappach The Yeast Arr4p ATPase Binds the Chloride Transporter Gef1p When Copper Is Available in the Cytosol J. Biol. Chem., January 6, 2006; 281(1): 410 - 417. [Abstract] [Full Text] [PDF]
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L. P. Cid, M. I. Niemeyer, and F. V. Sepulveda ClC-2 channels get new partners. Focus on "Association between Hsp90 and the ClC-2 chloride channel upregulates channel function" Am J Physiol Cell Physiol, January 1, 2006; 290(1): C42 - C44. [Full Text] [PDF]
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A. Hinzpeter, J. Lipecka, F. Brouillard, M. Baudoin-Legros, M. Dadlez, A. Edelman, and J. Fritsch Association between Hsp90 and the ClC-2 chloride channel upregulates channel function Am J Physiol Cell Physiol, January 1, 2006; 290(1): C45 - C56. [Abstract] [Full Text] [PDF]
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A. Liantonio, A. Picollo, E. Babini, G. Carbonara, G. Fracchiolla, F. Loiodice, V. Tortorella, M. Pusch, and D. C. Camerino Activation and Inhibition of Kidney CLC-K Chloride Channels by Fenamates Mol. Pharmacol., January 1, 2006; 69(1): 165 - 173. [Abstract] [Full Text] [PDF]
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 M. Suzuki The Drosophila tweety family: molecular candidates for large-conductance Ca2+-activated Cl- channels Exp Physiol, January 1, 2006; 91(1): 141 - 147. [Abstract] [Full Text] [PDF]
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S. Ignoul and J. Eggermont CBS domains: structure, function, and pathology in human proteins Am J Physiol Cell Physiol, December 1, 2005; 289(6): C1369 - C1378. [Abstract] [Full Text] [PDF]
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J. A. de Santiago, K. Nehrke, and J. Arreola Quantitative Analysis of the Voltage-dependent Gating of Mouse Parotid ClC-2 Chloride Channel J. Gen. Physiol., November 28, 2005; 126(6): 591 - 603. [Abstract] [Full Text] [PDF]
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T. Takano, J. Kang, J. K. Jaiswal, S. M. Simon, J. H.-C. Lin, Y. Yu, Y. Li, J. Yang, G. Dienel, H. R. Zielke, et al. Receptor-mediated glutamate release from volume sensitive channels in astrocytes PNAS, November 8, 2005; 102(45): 16466 - 16471. [Abstract] [Full Text] [PDF]
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S. J. Park, C. M. Mckay, Y. Zhu, and J. D. Huizinga Volume-activated chloride currents in interstitial cells of Cajal Am J Physiol Gastrointest Liver Physiol, November 1, 2005; 289(5): G791 - G797. [Abstract] [Full Text] [PDF]
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S.-S. Zhou, J. Yang, Y.-Q. Li, L.-Y. Zhao, M. Xu, and Y.-F. Ding Effect of Cl- channel blockers on aconitine-induced arrhythmias in rat heart Exp Physiol, November 1, 2005; 90(6): 865 - 872. [Abstract] [Full Text] [PDF]
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R. Robert, C. Norez, and F. Becq Disruption of CFTR chloride channel alters mechanical properties and cAMP-dependent Cl- transport of mouse aortic smooth muscle cells J. Physiol., October 15, 2005; 568(2): 483 - 495. [Abstract] [Full Text] [PDF]
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 C. Hartzell, Z. Qu, I. Putzier, L. Artinian, L.-T. Chien, and Y. Cui Looking Chloride Channels Straight in the Eye: Bestrophins, Lipofuscinosis, and Retinal Degeneration Physiology, October 1, 2005; 20(5): 292 - 302. [Abstract] [Full Text] [PDF]
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M. D Duffield, G. Y Rychkov, A. H Bretag, and M. L Roberts Zinc inhibits human ClC-1 muscle chloride channel by interacting with its common gating mechanism J. Physiol., October 1, 2005; 568(1): 5 - 12. [Abstract] [Full Text] [PDF]
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S. Yamaguchi and T. Ishikawa Electrophysiological characterization of native Na+-HCO3- cotransporter current in bovine parotid acinar cells J. Physiol., October 1, 2005; 568(1): 181 - 197. [Abstract] [Full Text] [PDF]
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 A. J. Fuller, B. C. Hauschild, R. Gonzalez-Villalobos, M. S. Awayda, J. D. Imig, E. W. Inscho, and L. G. Navar Calcium and chloride channel activation by angiotensin II-AT1 receptors in preglomerular vascular smooth muscle cells Am J Physiol Renal Physiol, October 1, 2005; 289(4): F760 - F767. [Abstract] [Full Text] [PDF]
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B. Bennetts, G. Y. Rychkov, H.-L. Ng, C. J. Morton, D. Stapleton, M. W. Parker, and B. A. Cromer Cytoplasmic ATP-sensing Domains Regulate Gating of Skeletal Muscle ClC-1 Chloride Channels J. Biol. Chem., September 16, 2005; 280(37): 32452 - 32458. [Abstract] [Full Text] [PDF]
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G. Pena-Munzenmayer, M. Catalan, I. Cornejo, C. D. Figueroa, J. E. Melvin, M. I. Niemeyer, L. P. Cid, and F. V. Sepulveda 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., September 15, 2005; 118(18): 4243 - 4252. [Abstract] [Full Text] [PDF]
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S. Lambert and J. Oberwinkler Characterization of a proton-activated, outwardly rectifying anion channel J. Physiol., August 15, 2005; 567(1): 191 - 213. [Abstract] [Full Text] [PDF]
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F. C. Britton, G.-L. Wang, Z. M. Huang, L. Ye, B. Horowitz, J. R. Hume, and D. Duan Functional Characterization of Novel Alternatively Spliced ClC-2 Chloride Channel Variants in the Heart J. Biol. Chem., July 8, 2005; 280(27): 25871 - 25880. [Abstract] [Full Text] [PDF]
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O. Strauss The Retinal Pigment Epithelium in Visual Function Physiol Rev, July 1, 2005; 85(3): 845 - 881. [Abstract] [Full Text] [PDF]
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 J de Gracia, F Mata, A Alvarez, T Casals, S Gatner, M Vendrell, D de la Rosa, L Guarner, and E Hermosilla Genotype-phenotype correlation for pulmonary function in cystic fibrosis Thorax, July 1, 2005; 60(7): 558 - 563. [Abstract] [Full Text] [PDF]
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