Regulatory-auxiliary subunits of CLC chloride channel-transport proteins

The Journal of Physiology
Alejandro Barrallo-GimenoR Estévez

Abstract

The CLC family of chloride channels and transporters is composed by nine members, but only three of them, ClC-Ka/b, ClC-7 and ClC-2, have been found so far associated with auxiliary subunits. These CLC regulatory subunits are small proteins that present few common characteristics among them, both structurally and functionally, and their effects on the corresponding CLC protein are different. Barttin, a protein with two transmembrane domains, is essential for the membrane localization of ClC-K proteins and their activity in the kidney and inner ear. Ostm1 is a protein with a single transmembrane domain and a highly glycosylated N-terminus. Unlike the other two CLC auxiliary subunits, Ostm1 shows a reciprocal relationship with ClC-7 for their stability. The subcellular localization of Ostm1 depends on ClC-7 and not the other way around. ClC-2 is active on its own, but GlialCAM, a transmembrane cell adhesion molecule with two extracellular immunoglobulin (Ig)-like domains, regulates its subcellular localization and activity in glial cells. The common theme for these three proteins is their requirement for a proper homeostasis, since their malfunction leads to distinct diseases. We will review here their properties and their role i...Continue Reading

References

Nov 1, 1976·The Journal of Clinical Investigation·M Imai, J P Kokko
Jan 1, 1995·The Journal of Clinical Investigation·S UchidaF Marumo
Jul 19, 1994·Proceedings of the National Academy of Sciences of the United States of America·S KieferleT J Jentsch
Dec 29, 1995·The Journal of Biological Chemistry·K SteinmeyerT J Jentsch
Mar 2, 1999·The Journal of Physiology·M PuschT J Jentsch
Jun 1, 2000·The Journal of Biological Chemistry·S Waldegger, T J Jentsch
Apr 27, 2002·The Journal of Biological Chemistry·Keith NehrkeJames E Melvin
May 17, 2002·The Journal of Physiology·Jorge ArreolaJames E Melvin
Jul 12, 2002·Pflügers Archiv : European journal of physiology·Siegfried WaldeggerHannsjörg W Seyberth
Jul 26, 2002·Journal of the American Society of Nephrology : JASN·Katsuki KobayashiSei Sasaki
Feb 8, 2003·The Journal of Clinical Endocrinology and Metabolism·Nobuhiro MiyamuraEiichi Araki
May 23, 2003·Histochemistry and Cell Biology·Atsushi HayamaShinichi Uchida
Jun 27, 2003·Proceedings of the National Academy of Sciences of the United States of America·Thierry FischerMarilyn Gist Farquhar
Aug 23, 2003·Molecular and Cellular Neurosciences·Judit K MakaraHelmut Kettenmann
Feb 27, 2004·Nature·Alessio Accardi, Christopher Miller
Mar 27, 2004·The New England Journal of Medicine·Karl P SchlingmannSiegfried Waldegger
May 29, 2004·EMBO Reports·Alessandra PicolloMichael Pusch
Jun 2, 2004·EMBO Reports·Peying Fong
Jun 5, 2004·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Paola QuarelloUgo Ramenghi
Feb 12, 2005·The EMBO Journal·Dagmar KasperThomas J Jentsch
Feb 16, 2005·Annual Review of Physiology·Shinichi Uchida, Sei Sasaki
Apr 15, 2005·Journal of the American Society of Nephrology : JASN·Thomas J Jentsch
Jul 22, 2005·Nature·Alessandra Picollo, Michael Pusch

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Citations

Sep 16, 2015·The Journal of Physiology·Peying Fong
Sep 15, 2017·The Journal of Physiology·Héctor Gaitán-PeñasRaúl Estévez
Aug 3, 2018·Biological & Pharmaceutical Bulletin·Hisao YamamuraYuji Imaizumi
Jan 22, 2020·Human Molecular Genetics·Xabier Elorza-VidalRaúl Estévez
Feb 9, 2021·Frontiers in Cellular Neuroscience·Assumpció Bosch, Raúl Estévez

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