Abstract
Ion transport across organellar membranes is important for the functioning of all intracellular organelles including those of the endo-/lysosomal system, but is still relatively little understood. In humans, 5 of the 9 members of the CLC gene family of chloride transporting proteins (CLC-3 through −7) are localized on endo-/lysosomal membranes, where they mediate secondary active stoichiometrically coupled, highly electrogenic, 2 Cl- / 1 H+ antiport (the remaining 4 CLC members are passive Cl- channels localized to the plasma membrane in various cell types).
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