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Nephrol Dial Transplant (2002) 17: 1-3
© 2002 European Renal Association-European Dialysis and Transplant Association

Editorial Comments

Diversity within the CLC chloride channel family involved in inherited diseases: from plasma membranes to acidic organelles

Alain Vandewalle

Faculté de Médecine, Xavier Bichat, Paris, France

Keywords: CLC chloride channels

Introduction

Since the first voltage-gated ClC-0 chloride channel was discovered by Jentsch et al. [1] in the marine ray Torpedo marmorata, nine chloride channels belonging to the CLC family have been identified in mammals [2]. These ClC channels exhibit marked differences in their tissue and cellular distribution. All ClC channels, except the ClC-1 channel [2], are expressed in mammalian kidneys. They have important functions, and mutations of some of them are responsible for inherited diseases. Furthermore, the development of ClC knockout mice models has produced a great deal of new information about the function(s) of these ClC channels and their pathological impact.

Expression and function of the kidney-specific ClC-K channels

Two closely homologous ClC chloride channels, referred to as rClC-K1 and rClC-K2 in rats and as hClC-Ka and hClC-Kb in humans [3,4], are almost exclusively expressed in kidney . . . [Full Text of this Article]

Expression and function of the ClC-5 channel in Dent's disease

Expression and function of the other ClC channels

Conclusions

Notes

References


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