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Article

Endosomal chloride/proton exchangers need inhibitory TMEM9 β-subunits for regulation and prevention of disease-causing overactivity.

Nature communications - 1 Apr 2025

Planells-Cases Rosa, Vorobeva Viktoriia, Kar Sumanta, Schmitt Franziska W, Schulte Uwe, Schrecker Marina, Hite Richard K, Fakler Bernd, Jentsch Thomas J

Abstract excerpt

The function of endosomes critically depends on their ion homeostasis. A crucial role of luminal Cl-, in addition to that of H+, is increasingly recognized. Both ions are transported by five distinct endolysosomal CLC chloride/proton exchangers. Dysfunction of each of these transporters entails severe disease. Here we identified TMEM9 and TMEM9B as obligatory β-subunits for endosomal ClC-3, ClC-4, and ClC-5. Mice...

Read the complete abstract on PubMed

Topics

Animals
Endosomes
Chloride Channels
Humans
Mice
Anoctamins

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Chlorides

Mice, Knockout

HEK293 Cells

Ion Transport

Lysosomes

Mutation

Source

Open in PubMed Open full text

Identifiers

PMID: 40169677
DOI: 10.1038/s41467-025-58546-3
PMCID: PMC11962092

Genes

TMEM9
CLC
TMEM9B

Provenance

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