The Cl-/H+ antiporter ClC-7 is the primary chloride permeation pathway in lysosomes

Nature

Volumn 453, Issue 7196, 2008, Pages 788-792

  Graves, Austin R ^a   Curran, Patricia K ^a   Smith, Carolyn L ^a   Mindell, Joseph A ^a  

^a NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ANTIPORTER; ANTIPORTER CIC 7; CHLORIDE ION; PROTON; SMALL INTERFERING RNA;

ACIDIFICATION; ANION; BIODEGRADATION; CELL ORGANELLE; CHLORIDE; ENZYME ACTIVITY; EXPERIMENTAL STUDY; MAMMAL; MOLECULAR ANALYSIS; PH; PROTEIN; RNA;

ACIDIFICATION; ANIMAL CELL; ANION TRANSPORT; ARTICLE; CELL MEMBRANE PERMEABILITY; CHLORIDE TRANSPORT; CONTROLLED STUDY; FEMALE; HELA CELL; HUMAN; HUMAN CELL; KNOCKOUT GENE; LIVER CELL; LYSOSOME; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN FUNCTION; RAT;

MAMMALIA;

EID: 44849107047     PISSN: 00280836     EISSN: 14764687     Source Type: Journal    
DOI: 10.1038/nature06907     Document Type: Article

References (22)

1. Dell'Antone, P. Evidence for an ATP-driven 'proton pump' in rat liver lysosomes by basic dyes uptake. Biochem. Biophys. Res. Commun. 86, 180-189 (1979).
2. Ohkuma, S., Moriyama, Y. & Takano, T. Identification and characterization of a proton pump on lysosomes by fluorescein-isothiocyanate- dextran fluorescence. Proc. Natl Acad. Sci. USA 79, 2758-2762 (1982).
3. Kornak, U. et al. Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man. Cell 104, 205-215 (2001).
4. White, M. M. & Miller, C. Avoltage-gated anion channel from the electric organ of Torpedo californica. J. Biol. Chem. 254, 10161-10166 (1979).
5. Picollo, A. & Pusch, M. Chloride/proton antiporter activity of mammalian CLC proteins ClC-4 and ClC-5. Nature 436, 420-423 (2005).
6. Scheel, O., Zdebik, A. A., Lourdel, S. & Jentsch, T. J. Voltage-dependent electrogenic chloride/proton exchange by endosomal CLC proteins. Nature 436, 424-427 (2005).
7. Graham, J. M. in Current Protocols in Cell Biology (eds Bonifacino, J. S., Dasso, M., Harford, J. B., Lippincott-Schwartz, J. & Yamada, K. M.) 3.6.1-3.6.21 (Wiley, Hoboken, NJ, 2000).
8. Goldberg, A. F. & Miller, C. Solubilization and functional reconstitution of a chloride channel from Torpedo californica electroplax. J. Membr. Biol. 124, 199-206 (1991).
9. Garty, H., Rudy, B. & Karlish, S. J. A simple and sensitive procedure for measuring isotope fluxes through ion-specific channels in heterogenous populations of membrane vesicles. J. Biol. Chem. 258, 13094-13099 (1983).
10. Rychkov, G. Y., Pusch, M., Roberts, M. L., Jentsch, T. J. & Bretag, A. H. Permeation and block of the skeletal muscle chloride channel, ClC-1, by foreign anions. J. Gen. Physiol. 111, 653-665 (1998).
11. Jentsch, T. J., Friedrich, T., Schriever, A. & Yamada, H. The CLC chloride channel family. Pflugers Arch. 437, 783-795 (1999).
12. Maduke, M., Pheasant, D. J. & Miller, C. High-level expression, functional reconstitution, and quaternary structure of a prokaryotic ClC-type chloride channel. J. Gen. Physiol. 114, 713-722 (1999).
13. Accardi, A. & Miller, C. Secondary active transport mediated by a prokaryotic homologue of ClC Cl2 channels. Nature 427, 803-807 (2004).
14. Illsley, N. P. & Verkman, A. S. Membrane chloride transport measured using a chloride-sensitive fluorescent probe. Biochemistry 26, 1215-1219 (1987).
15. Henriksen, K. et al. Characterization of osteoclasts from patients harboring a G215R mutation in ClC-7 causing autosomal dominant osteopetrosis type II. Am. J. Pathol. 164, 1537-1545 (2004).
16. Letizia, C. et al. Type II benign osteopetrosis (Albers-Schonberg disease) caused by a novel mutation in CLCN7 presenting with unusual clinical manifestations. Calcif. Tissue Int. 74, 42-46 (2004).
17. Kornak, U., Ostertag, A., Branger, S., Benichou, O. & de Vernejoul, M. C. 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 (2006).
18. Kasper, D. et al. Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration. EMBO J. 24, 1079-1091 (2005).
19. De Angeli, A. et al. The nitrate/proton antiporter AtCLCa mediates nitrate accumulation in plant vacuoles. Nature 442, 939-942 (2006).
20. Jentsch, T. J. Chloride and the endosomal-lysosomal pathway: emerging roles of CLC chloride transporters. J. Physiol. (Lond.) 578, 633-640 (2007).
21. Schaller, S. et al. The chloride channel inhibitor NS3736 prevents bone resorption in ovariectomized rats without changing bone formation. J. Bone Miner. Res. 19, 1144-1153 (2004).
22. Gottlieb, R. A. & Adachi, S. Nitrogen cavitation for cell disruption to obtain mitochondria from cultured cells. Methods Enzymol. 322, 213-221 (2000).