CLC5 is a member of the voltage-dependent Cl- channel family that includes nine known members. Based on sequence similarity, CLC5 is closely linked to the CLC3 and CLC4 subunits. Mutations in the CLC5 gene cause Dent's disease a X-linked renal disorder. CLC-5 is mainly found in intracellular vesicles,particularly in early endosomes vesicles, where it is believed to contribute to their acidification. The channel is mainly expressed in the kidney, although it has been found also in small intestine, brain and liver.