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Article

Uncoupling endosomal CLC chloride/proton exchange causes severe neurodegeneration.

The EMBO journal - 4 May 2020

Weinert Stefanie, Gimber Niclas, Deuschel Dorothea, Stuhlmann Till, Puchkov Dmytro, Farsi Zohreh, Ludwig Carmen F, Novarino Gaia, López-Cayuqueo Karen I, Planells-Cases Rosa, Jentsch Thomas J

Abstract excerpt

CLC chloride/proton exchangers may support acidification of endolysosomes and raise their luminal Cl- concentration. Disruption of endosomal ClC-3 causes severe neurodegeneration. To assess the importance of ClC-3 Cl- /H+ exchange, we now generate Clcn3unc/unc mice in which ClC-3 is converted into a Cl- channel. Unlike Clcn3-/- mice, Clcn3unc/unc mice appear normal owing to compensation by ClC-4 with which ClC-3...

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Identifiers

PMID: 32118314
DOI: 10.15252/embj.2019103358
PMCID: PMC7196918

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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