期刊
ACTA PHYSIOLOGICA SCANDINAVICA
卷 177, 期 2, 页码 119-147出版社
WILEY
DOI: 10.1046/j.1365-201X.2003.01060.x
关键词
Ca2+ activated Cl- channels; Cl- channel pathophysiology; ClC family; cystic firbrosis transmembrane conductance regulator; volume-activated Cl- channels
类别
Aim: 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.
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