Epithelial Anion Transport in Health and Disease

Cl absorption and HCO3 secretion are intimately associated processes vital to epithelial function, itself a key physiological activity. Until recently the transporters responsible remained obscure, but a breakthrough occurred with the discovery of the SLC26 transporters family.

Informationen zum Autor The Novartis Foundation is an international scientific and educational charity which promotes the study and general knowledge of science and in particular encourages international co-operation in scientific research. Klappentext Cl- absorption and HCO3- secretion are intimately associated processes vital to epithelial function, itself a key physiological activity. Until recently the transporters responsible remained obscure, but a breakthrough occurred with the discovery of the SLC26 transporters family. It is now clear that the SLC26 transporters have broad physiological functions since mutations in several members are linked to a variety of diseases. This book describes the properties of this family in detail, with contributions from the leading global researchers in the field. Complementary views from experts on other ion channels are offered in the discussions, which make fascinating reading.This family consists of at least 10 genes, each of which has several splice variants. Most members of the family are expressed in the luminal membrane of epithelial cells. Characterization of anion transport by three members has revealed that all function as Cl-/HCO3- exchangers, suggesting that SLC26 transporters are responsible for the luminal Cl-/HCO3- exchange activity. The SLC26 transporters are activated by the CF transmembrane conductance regulator and activate it in turn, leading to a model in which these molecules act together to mediate epithelial Cl- absorption and HCO3- secretion.The book includes chapters on the transport of other molecules by the SLC26 family, including oxalate in the kidney and sugars in cochlear hair cells amongst others. It also describes recent discoveries that most SLC26 transporters bind to scaffold proteins and that they all contain a conserved domain predicted to participate in protein-protein interactions. These suggest the SLC26 transporters exist in complexes with other Cl- and HCO3- transporters, and possibly other regulatory proteins. This book explores the functional role of these interactions, leading to better understanding of transepithelial fluid and electrolyte secretion and the diseases associated with it. Zusammenfassung Cl- absorption and HCO3- secretion are intimately associated processes vital to epithelial function, itself a key physiological activity. Until recently the transporters responsible remained obscure, but a breakthrough occurred with the discovery of the SLC26 transporters family. Inhaltsverzeichnis Michael Welsh Chair's introduction.Juha Kere Overview of the SLC26 family and associated diseases.Discussion.Marlies Knipper, Thomas Weber, Harald Winter, Claudia Braig, Jelka Cimerman, Juergen T. Fraenzer and Ulrike Zimmermann Individual characteristics of members of the SLC26 family in Vertebrates and their homologues in insects.Discussion.Daniel Markovich Sulfate transport by SLC26 transporters.Discussion.Jonathan Ashmore and Jean-Marie Chambard Sugar transport by members of the SLC26 superfamily.Discussion.Pia Höglund SLC26A3 and congenital chloride diarrhoea.Discussion.Manoocher Soleimani Expression, regulation and the role of SLC26 Cl-/HCO3- exchangers in kidney and gastrointestinal tract.Discussion.Seth L. Alper, Andrew K. Stewart, Marina N. Chernova,Alexander S. Zolotarev, Jeffrey S. Clark and David H. Vandorpe Anion exchangers in flux: functional differences between human and mouse SLC26A6 polypeptides.Discussion.Michael F. Romero, Min-Hwang Chang, Consuelo Plata,Kambiz Zandi-Nejad, Vadjista Broumand, Caroline R. Sussman and David B. Mount Physiology of electrogenic SLC26 paralogues.Discussion.Peter S. Aronson Role of SLC26-mediated Cl-/base exchange in proximal tubule naCl transport.Discussion.Péter Hegyi, Zoltán Rakonczay Jr., László Tiszlavica, András Varró,András Tóth, Gábor Rácz, Gábor Varga, Michael A. Gray and Barry E. Argent SLC26 ...

Autorentext
The Novartis Foundation is an international scientific and educational charity which promotes the study and general knowledge of science and in particular encourages international co-operation in scientific research.

Klappentext
Cl- absorption and HCO3- secretion are intimately associated processes vital to epithelial function, itself a key physiological activity. Until recently the transporters responsible remained obscure, but a breakthrough occurred with the discovery of the SLC26 transporters family. It is now clear that the SLC26 transporters have broad physiological functions since mutations in several members are linked to a variety of diseases. This book describes the properties of this family in detail, with contributions from the leading global researchers in the field. Complementary views from experts on other ion channels are offered in the discussions, which make fascinating reading. This family consists of at least 10 genes, each of which has several splice variants. Most members of the family are expressed in the luminal membrane of epithelial cells. Characterization of anion transport by three members has revealed that all function as Cl-/HCO3- exchangers, suggesting that SLC26 transporters are responsible for the luminal Cl-/HCO3- exchange activity. The SLC26 transporters are activated by the CF transmembrane conductance regulator and activate it in turn, leading to a model in which these molecules act together to mediate epithelial Cl- absorption and HCO3- secretion. The book includes chapters on the transport of other molecules by the SLC26 family, including oxalate in the kidney and sugars in cochlear hair cells amongst others. It also describes recent discoveries that most SLC26 transporters bind to scaffold proteins and that they all contain a conserved domain predicted to participate in protein-protein interactions. These suggest the SLC26 transporters exist in complexes with other Cl- and HCO3- transporters, and possibly other regulatory proteins. This book explores the functional role of these interactions, leading to better understanding of transepithelial fluid and electrolyte secretion and the diseases associated with it.

Inhalt
Michael Welsh Chair's introduction. Juha Kere Overview of the SLC26 family and associated diseases. Discussion. Marlies Knipper, Thomas Weber, Harald Winter, Claudia Braig, Jelka Cimerman, Juergen T. Fraenzer and Ulrike Zimmermann Individual characteristics of members of the SLC26 family in Vertebrates and their homologues in insects. Discussion. Daniel Markovich Sulfate transport by SLC26 transporters. Discussion. Jonathan Ashmore and Jean-Marie Chambard Sugar transport by members of the SLC26 superfamily. Discussion. Pia Höglund SLC26A3 and congenital chloride diarrhoea. Discussion. Manoocher Soleimani Expression, regulation and the role of SLC26 Cl-/HCO3- exchangers in kidney and gastrointestinal tract. Discussion. Seth L. Alper, Andrew K. Stewart, Marina N. Chernova,Alexander S. Zolotarev, Jeffrey S. Clark and David H. Vandorpe Anion exchangers in flux: functional differences between human and mouse SLC26A6 polypeptides. Discussion. Michael F. Romero, Min-Hwang Chang, Consuelo Plata,Kambiz Zandi-Nejad, Vadjista Broumand, Caroline R. Sussman and David B. Mount Physiology of electrogenic SLC26 paralogues. Discussion. Peter S. Aronson Role of SLC26-mediated Cl-/base exchange in proximal tubule naCl transport. Discussion. Péter Hegyi, Zoltán Rakonczay Jr., László Tiszlavica, András Varró,András Tóth, Gábor Rácz, Gábor Varga, Michael A. Gray and Barry E. Argent SLC26 transporters and the inhibitory control of pancreatic ductal bicarbonate secretion. Discussion. Nikolay Shcheynikov, Shigeru B. H. Ko, Weizhong …

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