CLC AND CFTR CHLORIDE CHANNEL GATING

Chloride channels are widely expressed and play important roles in cel l volume regulation, transepithelial transport, intracellular pH regul ation, and membrane excitability. Most chloride channels have yet to b e identified at a molecular level. The CIC gene family and the cystic fibrosis transmembrane conductance regulator (CFTR) are distinct chlor ide channels expressed in many cell types, and mutations in their gene s are the cause of several diseases including myotonias, cystic fibros is, and kidney stones. Because of their molecular definition and roles in disease, these channels have been studied intensively over the pas t several years. The focus of this review is on recent studies that ha ve provided new insights into the mechanisms governing the opening and closing, i.e. gating, of the ClC and CFTR chloride channels.