[Structure and function of ClC chloride channels].
ClC chloride channels form a voltage-gated chloride channel family. It consists of at least 8 members of chloride channel, including the skeletal muscle-specific ClC-1 and the two very homologous kidney-specific chloride channels, ClC-K1 and ClC-K2. The dysfunction of ClC-1 is shown to lead to myotonia. Both ClC-K1 and ClC-K2 are involved in the transcellular chloride transport in the different nephron segments and may be involved in certain disorders of tubular function. This review focuses on (1) the structure and function of ClC chloride channels and (2) the involvement of ClC chloride channels in human diseases.
Uchida
S
S
Second Department of Internal Medicine, Tokyo Medical and Dental University, School of Medicine, Japan.
D000818:Animals; D018118:Chloride Channels / Q000378:metabolism / Q000502:physiology; D002712:Chlorides / Q000378:metabolism*; D006801:Humans; D007668:Kidney / Q000378:metabolism; D007674:Kidney Diseases / Q000209:etiology; D018482:Muscle, Skeletal / Q000378:metabolism; D009222:Myotonia / Q000209:etiology
D018118:Chloride Channels; D002712:Chlorides