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Article

Impaired Autophagic Clearance with a Gain-of-Function Variant of the Lysosomal Cl-/H+ Exchanger ClC-7.

Biomolecules - 15 Dec 2023

Bose Shroddha, de Heus Cecilia, Kennedy Mary E, Wang Fan, Jentsch Thomas J, Klumperman Judith, Stauber Tobias

Abstract excerpt

ClC-7 is a ubiquitously expressed voltage-gated Cl-/H+ exchanger that critically contributes to lysosomal ion homeostasis. Together with its β-subunit Ostm1, ClC-7 localizes to lysosomes and to the ruffled border of osteoclasts, where it supports the acidification of the resorption lacuna. Loss of ClC-7 or Ostm1 leads to osteopetrosis accompanied by accumulation of storage material in lysosomes and...

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Identifiers

PMID: 38136669
DOI: 10.3390/biom13121799
PMCID: PMC10742274

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