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Article

ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function.

Nature - 9 Mar 2006

Lange Philipp F, Wartosch Lena, Jentsch Thomas J, Fuhrmann Jens C

Abstract excerpt

Mutations in ClC-7, a late endosomal/lysosomal member of the CLC family of chloride channels and transporters, cause osteopetrosis and lysosomal storage disease in humans and mice. Severe osteopetrosis is also observed with mutations in the OSTM1 gene, which encodes a membrane protein of unknown...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 16525474
DOI: 10.1038/nature04535

Genes

CLC
OSTM1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-16525474-2026-09-02.2. Manifest publication-graph-cohort-2026-09-02.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 2 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function.PMID 16525474Read ↗

Molecular insights into the human CLC-7/Ostm1 transporter.Semantic proximity Read ↗

Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC-7.Semantic proximity Read ↗

Transport activity and presence of ClC-7/Ostm1 complex account for different cellular functions.Semantic proximity Read ↗

The molecular and cellular interplay between the osteopetrosis-associated proteins SNX10, OSTM1, and CLC-7 during osteoclastogenesisSemantic proximity Read ↗

Pathobiologic Mechanisms of Neurodegeneration in Osteopetrosis Derived From Structural and Functional Analysis of 14 ClC-7 Mutants.Semantic proximity Read ↗

Loss of the chloride channel ClC-7 leads to lysosomal storage disease and neurodegeneration.Semantic proximity Read ↗

Characterization of the bone phenotype in ClC-7-deficient mice.Semantic proximity Read ↗

CLCN7, a gene shared by autosomal recessive and autosomal dominant osteopetrosis.Semantic proximity Read ↗

Null mutation of chloride channel 7 (Clcn7) impairs dental root formation but does not affect enamel mineralization.Semantic proximity Read ↗

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

A missense mutation accelerating the gating of the lysosomal Cl-/H+-exchanger ClC-7/Ostm1 causes osteopetrosis with gingival hamartomas in cattle.Semantic proximity Read ↗

ClC-7 expression levels critically regulate bone turnover, but not gastric acid secretion.Semantic proximity Read ↗

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