Hanyang Med Rev.  2008 Nov;28(4):4-10.

The properties of voltage-sensitive chloride channels

• Jung SJ

Abstract

Chloride (Cl) channels are probably found in every cell, from bacteria to mammals. Cl channels are distributed both in the plasma membrane and in intracellular organelles. Three well established structural classes of plasma membrane chloride channels now exist: the ligand-gated chloride channels, the cAMP-stimulated cystic fibrosis transmembrane conductance regulator channel, and the voltage-gated (or swelling-activated) members of the ClC chloride channel family. They have diverse functions, ranging from regulation of cell volume to transepithelial transport, homeostasis, and stabilization of membrane potential, signal transduction and acidification of intracellular organelles. These different functions require the presence of many distinct Cl channels, which are differentially expressed and regulated by various stimuli. A combination of mutagenesis and biophysical analysis has been used to correlate their structure with function. Also their physiological roles are explained by genetic defects leading to various inherited disease and knock-out mouse models. Thus, the loss of Cl channels leads to an impairment of transepithelial transport in Bartter's syndrome, to increased excitability in congenital myotonia, and to reduced endosomal acidification and impaired endocytosis in Dent's disease. Three major structural classes of chloride channels are known to date, but there may be others not yet identified at the molecular level. This review focuses on voltage-gated members of the ClC chloride channel family and their physiological roles.

Keyword

Chloride; Ion channel; Voltage-sensitive chloride channel; Genetic disorder; Excitability

MeSH Terms

Animals
Bacteria
Bartter Syndrome
Cell Membrane
Cell Size
Chloride Channels
Cystic Fibrosis Transmembrane Conductance Regulator
Dent Disease
Endocytosis
Homeostasis
Humans
Ion Channels
Mammals
Membrane Potentials
Mice
Mutagenesis
Myotonia
Organelles
Signal Transduction
Chloride Channels
Cystic Fibrosis Transmembrane Conductance Regulator
Ion Channels