Diversity within the CLC chloride channel family involved in inherited diseases: from plasma membranes to acidic organelles.

Since the first voltage-gated ClC-0 chloride channel was discovered by Jentsch et al. w1x in the marine ray Torpedo marmorata, nine chloride channels belonging to the CLC family have been identified in mammals w2x. These ClC channels exhibit marked differences in their tissue and cellular distribution. All ClC channels, except the ClC-1 channel w2x, are expressed in mammalian kidneys. They have important functions, and mutations of some of them are responsible for inherited diseases. Furthermore, the development of ClC knockout mice models has produced a great deal of new information about the function(s) of these ClC channels and their pathological impact.