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Article

Lysosomal pathology and osteopetrosis upon loss of H+-driven lysosomal Cl- accumulation.

Science (New York, N.Y.) - 11 Jun 2010

Weinert Stefanie, Jabs Sabrina, Supanchart Chayarop, Schweizer Michaela, Gimber Niclas, Richter Martin, Rademann Jörg, Stauber Tobias, Kornak Uwe, Jentsch Thomas J

Abstract excerpt

During lysosomal acidification, proton-pump currents are thought to be shunted by a chloride ion (Cl-) channel, tentatively identified as ClC-7. Surprisingly, recent data suggest that ClC-7 instead mediates Cl-/proton (H+) exchange. We generated mice carrying a point mutation converting ClC-7 int...

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Identifiers

PMID: 20430974
DOI: 10.1126/science.1188072

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 2 Sept 2026. Prepared record publication-20430974-2026-09-02.2. 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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Lysosomal pathology and osteopetrosis upon loss of H+-driven lysosomal Cl- accumulation.PMID 20430974Read ↗

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

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

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

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

Uncoupling endosomal CLC chloride/proton exchange causes severe neurodegeneration.Semantic proximity Read ↗

Large transient capacitive currents in wild-type lysosomal Cl-/H+ antiporter ClC-7 and residual transport activity in the proton glutamate mutant E312A.Semantic proximity Read ↗

A model of lysosomal pH regulation.Semantic proximity Read ↗

Multisystem disorder associated with a pathogenic variant in CLCN7 in the absence of osteopetrosis.Semantic proximity Read ↗

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

Tonic inhibition of the chloride/proton antiporter ClC-7 by PI(3,5)P2 is crucial for lysosomal pH maintenanceSemantic proximity Read ↗

[Function of the CLC chloride channels and their implication in human pathology].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 ↗

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