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. 2015 May 19;6:159. doi: 10.3389/fphys.2015.00159

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Copyright © 2015 Alekov.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Cellular localization and transport of WT and disease-associated ClC-5 mutants. (A–C) Confocal florescent images of cells transfected with either WT or the mutants G212A and E267A ClC-5 with fused mCherry at the C-terminus. Visible as bright fluorescent spots is the strong vesicular localization but a significant percentage of the ClC-5 proteins are also localized to the plasma membrane. (D–F) Current families recorded from cells expressing the investigated constructs by whole-cell patch-clamp upon voltage steps between −115 and +175 mV. The insets depict enlarged the off-gating currents for the corresponding mutant that have been used subsequently for estimating the voltage-dependence of ClC-5 activation.