CLC chloride channels and transporters

被引:106
作者
Jentsch, TJ [1 ]
Neagoe, L [1 ]
Scheel, O [1 ]
机构
[1] Univ Hamburg, ZMNH, Zentrum Mol Neurobiol, D-20246 Hamburg, Germany
关键词
D O I
10.1016/j.conb.2005.05.002
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
CLC proteins are found in cells from prokaryotes to mammals and perform functions in plasma membranes and intracellular vesicles. Several genetic human diseases and mouse models underscore their broad physiological functions in mammals. These functions range from the control of excitability to transepithelial transport, endocytotic trafficking and acidification of synaptic vesicles. The recent crystallization of bacterial CLC proteins gave surprising insights into CLC Cl--channel permeation and gating and provides an excellent basis for structure-function studies. Surprisingly, the CLC from Escherichia coli functions as a Cl-/H+ exchanger, thus demonstrating the thin line separating transporters and channels.
引用
收藏
页码:319 / 325
页数:7
相关论文
共 55 条
[1]   Conformational changes in the pore of CLC-0 [J].
Accardi, A ;
Pusch, M .
JOURNAL OF GENERAL PHYSIOLOGY, 2003, 122 (03) :277-293
[2]   Secondary active transport mediated by a prokaryotic homologue of ClC Cl- channels [J].
Accardi, A ;
Miller, C .
NATURE, 2004, 427 (6977) :803-807
[3]   Mutation of BSND causes Bartter syndrome with sensorineural deafness and kidney failure [J].
Birkenhäger, R ;
Otto, E ;
Schürmann, 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, NVAM ;
Antignac, C ;
Sudbrak, R ;
Kispert, A ;
Hildebrandt, F .
NATURE GENETICS, 2001, 29 (03) :310-314
[4]  
Bosi M.R., 2001, EMBO J, V20, P1289
[5]   ClC-2 in guinea pig colon:: mRNA, immunolabeling, and functional evidence for surface epithelium localization [J].
Catalán, M ;
Cornejo, I ;
Figueroa, CD ;
Niemeyer, MI ;
Sepulveda, FV ;
Cid, LP .
AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY, 2002, 283 (04) :G1004-G1013
[6]   Loss of chloride channel ClC-5 impairs endocytosis by defective trafficking of megalin and cubilin in kidney proximal tubules [J].
Christensen, EI ;
Devuyst, O ;
Dom, G ;
Nielsen, R ;
Van Der Smissen, P ;
Verroust, P ;
Leruth, M ;
Guggino, WB ;
Courtoy, PJ .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2003, 100 (14) :8472-8477
[7]   Albers-Schonberg disease (autosomal dominant osteopetrosis, type II) results from mutations in the CICN7chloride channel gene [J].
Cleiren, E ;
Bénichou, O ;
Van Hul, E ;
Gram, J ;
Bollerslev, J ;
Singer, FR ;
Beaverson, K ;
Aledo, A ;
Whyte, MP ;
Yoneyama, T ;
deVernejoul, MC ;
Van Hul, W .
HUMAN MOLECULAR GENETICS, 2001, 10 (25) :2861-2867
[8]   Mechanism of anionic conduction across ClC [J].
Cohen, J ;
Schulten, K .
BIOPHYSICAL JOURNAL, 2004, 86 (02) :836-845
[9]  
D'Agostino D, 2004, NEUROLOGY, V63, P1500
[10]   X-ray structure of a CIC chloride channel at 3.0 Å reveals the molecular basis of anion selectivity [J].
Dutzler, R ;
Campbell, EB ;
Cadene, M ;
Chait, BT ;
MacKinnon, R .
NATURE, 2002, 415 (6869) :287-294