Conformational changes in the pore of CLC-0

Journal of General Physiology

Volumn 122, Issue 3, 2003, Pages 277-293

  Accardi, Alessio ^a,b   Pusch, Michael ^a  

^a CNR   (Italy)

^b BRANDEIS UNIVERSITY   (United States)

Author keywords

Chloride channel; CLC; Clofibric acid; Double barreled; Fast gate

Indexed keywords

4 CHLOROPHENOXYACETIC ACID; ALANINE; CHLORIDE CHANNEL; CLOFIBRIC ACID DERIVATIVE; PHENYLALANINE; TYROSINE;

AMINO ACID SUBSTITUTION; ARTICLE; BINDING AFFINITY; BINDING SITE; CHANNEL GATING; CONFORMATIONAL TRANSITION; CRYSTAL STRUCTURE; DISSOCIATION; ELECTRIC POTENTIAL; KINETICS; MATHEMATICAL MODEL; MEMBRANE CONDUCTANCE; MEMBRANE CURRENT; MUTANT; MUTATION;

2,4-DICHLOROPHENOXYACETIC ACID; ANIMALS; BINDING SITES; CHLORIDE CHANNELS; CHLORIDES; ELECTRIC CONDUCTIVITY; EXTRACELLULAR FLUID; ION CHANNEL GATING; KINETICS; MODELS, BIOLOGICAL; MODELS, CHEMICAL; MOLECULAR CONFORMATION; MUTATION; OOCYTES; OSMOLAR CONCENTRATION; TORPEDO; XENOPUS;

EID: 0041899029     PISSN: 00221295     EISSN: None     Source Type: Journal    
DOI: 10.1085/jgp.200308834     Document Type: Article

References (34)

1. Accardi, A., L. Ferrera, and M. Pusch. 2001. Drastic reduction of the slow gate of human muscle chloride channel (ClC-1) by mutation C277S. J. Physiol. 534:745-752.
2. Accardi, A., and M. Pusch. 2000. Fast and slow gating relaxations in the muscle chloride channel CLC-1. J. Gen. Physiol. 116:433-444.
3. Aromataris, E.C., D.S. Astill, G.Y. Rychkov, S.H. Bryant, A.H. Bretag, and M.L. Roberts. 1999. Modulation of the gating of ClC-1 by S-(-) 2-(4-chlorophenoxy) propionic acid. Br. J. Pharmacol. 126:1375-1382.
4. Bösl, M.R., V. Stein, C. Hübner, A.A. Zdebik, S.E. Jordt, A.K. Mukhopadhyay, M.S. Davidoff, A.F. Holstein, and T.J. Jentsch. 2001. Male germ cells and photoreceptors, both dependent on close cell-cell interactions, degenerate upon ClC-2 Cl(-) channel disruption. EMBO J. 20:1289-1299.
5. Chen, M., and T. Chen. 2002. Side-chain charge effect on the channel conductance of CLC-0. Biophys. J. 82:12d.
6. Chen, M.F., and T.Y. Chen. 2001. Different fast-gate regulation by external Cl(-) and H(+) of the muscle-type ClC chloride channels. J. Gen. Physiol. 118:23-32.
7. - channel. J. Gen. Physiol. 108:237-250.
8. Conte-Camerino, D., M. Mambrini, A. DeLuca, D. Tricarico, S.H. Bryant, V. Tortorella, and G. Bettoni. 1988. Enantiomers of clofibric acid analogs have opposite actions on rat skeletal muscle chloride channels. Pflugers Arch. 413:105-107.
9. + conduction and selectivity. Science. 280:69-77.
10. Dutzler, R., E.B. Campbell, M. Cadene, B.T. Chait, and R. MacKinnon. 2002. X-ray structure of a ClC chloride channel at 3.0 Å reveals the molecular basis of anion selectivity. Nature. 415:287-294.
11. Dutzler, R., E.B. Campbell, and R. MacKinnon. 2003. Gating the selectivity filter in ClC chloride channels. Science. 300:108-112.
12. Estévez, R., B.C. Schroeder, A. Accardi, T.J. Jentsch, and M. Pusch. 2003. Conservation of chloride channel structure revealed by an inhibitor binding site in ClC-1. Neuron. 38:47-59.
13. Hanke, W., and C. Miller. 1983. Single chloride channels from Torpedo electroplax. Activation by protons. J. Gen. Physiol. 82:25-45.
14. Humphrey, W., A. Dalke, and K. Schulten. 1996. VMD: visual molecular dynamics. J. Mol. Graph. 14:33-38.
15. Iyer, R., T.M. Iverson, A. Accardi, and C. Miller. 2002. A biological role for prokaryotic ClC chloride channels. Nature. 419:715-718.
16. Jentsch, T.J., V. Stein, F. Weinreich, and A.A. Zdebik. 2002. Molecular structure and physiological function of chloride channels. Physiol. Rev. 82:503-568.
17. Koch, M.C., K. Steinmeyer, C. Lorenz, K. Ricker, F. Wolf, M. Otto, B. Zoll, E Lehmann-Horn, K.H. Grzeschik, and T.J. Jentsch, 1992. The skeletal muscle chloride channel in dominant and recessive human myotonia. Science. 257:797-800.
18. Kornak, U., D. Kasper, M.R. Bösl, E. Kaiser, M. Schweizer, A. Schulz, W. Friedrich, G. Delling, and T.J. Jentsch. 2001. Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man. Cell. 104:205-215.
19. Lin, C.W., and T.Y. Chen. 2000. Cysteine modification of a putative pore residue in ClC-0: implication for the pore stoichiometry of ClC chloride channels. J. Gen. Physiol. 116:535-546.
20. Lin, Y.W., C.W. Lin, and T.Y. Chen. 1999. Elimination of the slow gating of ClC-0 chloride channel by a point mutation. J. Gen. Physiol. 114:1-12.
21. Lloyd, S.E., S.H. Pearce, S.E. Fisher, K. Steinmeyer, B. Schwappach, S.J. Scheinman, B. Harding, A. Bolino, M. Devoto, P. Goodyer, et al. 1996. A common molecular basis for three inherited kidney stone diseases. Nature. 379:445-449.
22. Ludewig, U., T.J. Jentsch, and M. Pusch. 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.
23. Ludewig, U., M. Pusch, and T.J. Jentsch. 1996. Two physically distinct pores in the dimeric ClC-0 chloride channel. Nature. 383: 340-343.
24. Ludewig, U., M. Pusch, and T.J. Jentsch. 1997b. Independent gating of single pores in CLC-0 chloride channels. Biophys. J. 73: 789-797.
25. Maduke, M., C. Miller, and J.A. Mindell. 2000. A decade of CLC chloride channels: structure, mechanism, and many unsettled questions. Annu. Rev. Biophys. Biomol. Struct. 29:411-438.
26. Middleton, R.E., D.J. Pheasant, and C. Miller. 1996. Homodimeric architecture of a ClC-type chloride ion channel. Nature. 383:337-340.
27. --channel disruption impairs endocytosis in a mouse model for Dent's disease. Nature. 408:369-373.
28. Pusch, M., A. Accardi, A. Liantonio, L. Ferrera, A. De Luca, D.C. Camerino, and F. Conti. 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.
29. Pusch, M., A. Liantonio, L. Bertorello, A. Accardi, A. De Luca, S. Pierno, V. Tortorella, and D.C. Camerino. 2000. Pharmacological characterization of chloride channels belonging to the ClC family by the use of chiral clofibric acid derivatives. Mol. Pharmacol. 58:498-507.
30. Pusch, M., U. Ludewig, A. Rehfeldt, and T.J. Jentsch. 1995. Gating of the voltage-dependent chloride channel ClC-0 by the permeant anion. Nature. 373:527-531.
31. Saviane, C., F. Conti, and M. Pusch. 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.
32. Simon, D.B., R.S. Bindra, T.A. Mansfield, C. Nelson-Williams, E. Mendonca, R. Stone, S. Schurman, A. Nayir, H. Alpay, A. Bakkaloglu, et al. 1997. Mutations in the chloride channel gene, CLCNKB, cause Bartter's syndrome type III. Nat. Genet. 17:171-178.
33. Stobrawa, S.M., T. Breiderhoff, S. Takamori, D. Engel, M. Schweizer, A.A. Zdebik, M.R. Bösl, K. Ruether, H. Jahn, A. Draguhn, et al. 2001. Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus. Neuron. 29: 185-196.
34. Traverso, S., L. Elia, and M. Pusch. 2003. Gating competence of constitutively open CLC-0 mutants revealed by the interaction with a small organic inhibitor. In press.