CLC Chloride Channels and Transporters: Structure, Function, Physiology, and Disease

Physiological Reviews
Thomas J Jentsch, M Pusch

Abstract

CLC anion transporters are found in all phyla and form a gene family of eight members in mammals. Two CLC proteins, each of which completely contains an ion translocation parthway, assemble to homo- or heteromeric dimers that sometimes require accessory β-subunits for function. CLC proteins come in two flavors: anion channels and anion/proton exchangers. Structures of these two CLC protein classes are surprisingly similar. Extensive structure-function analysis identified residues involved in ion permeation, anion-proton coupling and gating and led to attractive biophysical models. In mammals, ClC-1, -2, -Ka/-Kb are plasma membrane Cl- channels, whereas ClC-3 through ClC-7 are 2Cl-/H+-exchangers in endolysosomal membranes. Biological roles of CLCs were mostly studied in mammals, but also in plants and model organisms like yeast and Caenorhabditis elegans. CLC Cl- channels have roles in the control of electrical excitability, extra- and intracellular ion homeostasis, and transepithelial transport, whereas anion/proton exchangers influence vesicular ion composition and impinge on endocytosis and lysosomal function. The surprisingly diverse roles of CLCs are highlighted by human and mouse disorders elicited by mutations in their ge...Continue Reading

References

Mar 1, 1977·The Journal of General Physiology·P T Palade, R L Barchi
May 15, 1979·The Journal of Comparative Neurology·G Q Fox, G P Richardson
Jun 1, 1978·The Journal of Comparative Neurology·G Q Fox, G P Richardson
Oct 1, 1978·Annals of Neurology·R E Furman, R L Barchi
Jun 1, 1977·The Journal of General Physiology·P T Palade, R L Barchi
Jun 1, 1991·Pflügers Archiv : European journal of physiology·D TricaricoS H Bryant
Dec 15, 1991·Proceedings of the National Academy of Sciences of the United States of America·C K BauerT J Jentsch
Jan 1, 1991·Annual Review of Physiology·S L Alper
Jan 1, 1986·Pflügers Archiv : European journal of physiology·P WangemannR Greger
Apr 1, 1987·Physiological Reviews·A H Bretag
Nov 13, 1987·Biochemical and Biophysical Research Communications·H Brinkmeier, H Jockusch
May 1, 1988·Muscle & Nerve·G MehrkeH Jockusch
Jun 1, 1988·Muscle & Nerve·H KwiecińskiR Rüdel
Nov 1, 1988·Pflügers Archiv : European journal of physiology·D Conte-CamerinoG Bettoni
Apr 1, 1985·Physiological Reviews·R Rüdel, F Lehmann-Horn
Mar 1, 1989·Pflügers Archiv : European journal of physiology·D Conte CamerinoG Vrbovà
Feb 1, 1988·Brain : a Journal of Neurology·S F BerkovicL S Wolfe
Jul 1, 1974·The Journal of Physiology·R H Adrian, S H Bryant
Dec 1, 1971·The Journal of Physiology·S H Bryant, A Morales-Aguilera
Dec 1, 1982·Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences·C Miller
Jan 1, 1980·Annals of the New York Academy of Sciences·C Miller, M M White
Jul 1, 1981·The Journal of General Physiology·M M White, C Miller
Dec 1, 1982·Proceedings of the National Academy of Sciences of the United States of America·D W TankW W Webb
Jul 1, 1983·The Journal of General Physiology·W Hanke, C Miller
May 1, 1984·Proceedings of the National Academy of Sciences of the United States of America·C Miller, M M White
May 1, 1995·The American Journal of Physiology·M GekleS Silbernagl
Aug 1, 1995·Human Molecular Genetics·F Lehmann-HornA L George
Aug 1, 1995·Nature Genetics·N A Ellis

❮ Previous
Next ❯

Citations

Jan 13, 2019·Journal of the American Society of Nephrology : JASN·Laura LagostenaAlessandra Picollo
Jun 10, 2019·The Journal of Physiology·Carla Pérez-RiusRaúl Estévez
Sep 12, 2019·Physiological Reviews·S F Pedersen, L Counillon
Dec 26, 2019·The Journal of General Physiology·Donald W Hilgemann
Feb 6, 2020·Bioinformatics·Rostislav K SkitchenkoAlbert Guskov
Mar 3, 2020·The EMBO Journal·Stefanie WeinertThomas J Jentsch
May 12, 2020·EMBO Reports·Matthias GrieschatAlexi K Alekov
Nov 8, 2019·Biochemical Society Transactions·Mauricio Di Fulvio, Lydia Aguilar-Bryan
Jun 5, 2020·American Journal of Hypertension·Fred S LambRyan J Stark
Sep 16, 2018·Journal of Hematology & Oncology·Zhuoyu GuLiwei Wang
Jun 30, 2019·SLAS Discovery·Mariafrancesca ScaliseCesare Indiveri
Oct 3, 2019·American Journal of Physiology. Cell Physiology·Viktor YarotskyyGeorgi V Petkov
Jun 18, 2020·Proceedings of the National Academy of Sciences of the United States of America·Elsa DemesAlexis De Angeli
Jun 26, 2020·Scientific Reports·Andrea GerbinoMonica Carmosino
Oct 17, 2019·Nature Communications·Corinna GöppnerThomas J Jentsch
Aug 15, 2019·Proceedings of the National Academy of Sciences of the United States of America·Kunwoong ParkHyun-Ho Lim
Jul 6, 2020·Pflügers Archiv : European journal of physiology·Dong Hoon ShinMin Goo Lee
May 4, 2020·Pflügers Archiv : European journal of physiology·Concetta AltamuraPaola Imbrici
Jul 30, 2020·Nature Reviews. Endocrinology·Maria-Christina ZennaroFabio L Fernandes-Rosa
Aug 29, 2020·Journal of Molecular Neuroscience : MN·Vladimir A Martínez-RojasCarlo Musio
Oct 6, 2020·Frontiers in Neurology·Concetta AltamuraJean-François Desaphy
Nov 7, 2019·American Journal of Physiology. Cell Physiology·Benjamin P LüscherShmuel Muallem
Dec 20, 2019·Traffic·Jean Gruenberg
Mar 3, 2020·Frontiers in Physiology·Shubha Gururaja RaoHarpreet Singh
Apr 24, 2020·Frontiers in Neuroscience·Bettina U WilkeMichaela Kress
Aug 28, 2020·Science Advances·Sensen ZhangMaojun Yang
Oct 6, 2020·Function·Katherine E Shipman, Ora A Weisz
Dec 19, 2019·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Young Su JooSeung Hyeok Han
Oct 10, 2020·Physiologia Plantarum·Ashish SubbaSneh L Singla-Pareek

❮ Previous
Next ❯

Methods Mentioned

BETA
X-ray
dissecting
FRET
NMR
isothermal titration calorimetry
co-crystallization
PCR
transfection
ubiquitination
glycosylation

Related Concepts

Related Feeds

Auditory Perception

Auditory perception is the ability to receive and interpret information attained by the ears. Here is the latest research on factors and underlying mechanisms that influence auditory perception.