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Chloride accumulation in endosomes and lysosomes: facts checked in mice
1Department of Molecular Biology, Universitätsmedizin Göttingen, Göttingen, Germany.
The EMBO Journal
|April 10, 2020
Abstract:
Loss of the ClC-3 chloride/proton exchanger found in intracellular compartments leads to marked neurodegeneration. New genetic work by Weinert et al (2020) now shows that selective impairment of ClC-3's ion exchange activity is sufficient to elicit this severe phenotype in vivo. ClC-3 cooperates with the closely related ClC-4 in protecting endolysosomal chloride balance and neuronal integrity.

