Amazing chloride channels: an overview.
This review describes molecular and functional properties of the following Cl- channels: the ClC family of voltage-dependent Cl- channels, the cAMP-activated transmembrane conductance regulator (CFTR), Ca2+ activated Cl- channels (CaCC) and volume-regulated anion channels (VRAC). If structural data are available, their relationship with the function of Cl- channels will be discussed. We also describe shortly some recently discovered channels, including high conductance Cl- channels and the family of bestrophins. We illustrate the growing physiological importance of these channels in the plasma membrane and in intracellular membranes, including their involvement in transepithelial transport, pH regulation of intracellular organelles, regulation of excitability and volume regulation. Finally, we discuss the role of Cl- channels in various diseases and describe the pathological phenotypes observed in knockout mice models.
Nilius
B
B
KU Leuven, Laboratorium voor Fysiologie, Campus Gasthuisberg, Leuven, Belgium.
Droogmans
G
G
D000818:Animals; D000838:Anions / Q000378:metabolism; D001692:Biological Transport; D002118:Calcium / Q000502:physiology; D002462:Cell Membrane / Q000502:physiology; D018118:Chloride Channels / Q000502:physiology*; D000242:Cyclic AMP / Q000502:physiology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000502:physiology; D004195:Disease Models, Animal; D004553:Electric Conductivity; D006863:Hydrogen-Ion Concentration; D007425:Intracellular Membranes / Q000502:physiology; D007700:Kinetics; D051379:Mice; D018345:Mice, Knockout; D008954:Models, Biological; D015388:Organelles / Q000502:physiology; D010641:Phenotype; D010766:Phosphorylation; D011506:Proteins / Q000378:metabolism
D000838:Anions; D018118:Chloride Channels; D011506:Proteins; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D000242:Cyclic AMP; D002118:Calcium