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CLCN7 protein, human

Known as: CLC-7 chloride channel protein, human, Chloride Channel 7 Alpha Subunit, CLCN7 
H(+)/Cl(-) exchange transporter 7 (805 aa, ~89 kDa) is encoded by the human CLCN7 gene. This protein plays a role in proton-coupled, chloride anion… 
National Institutes of Health

Related topics

Related topics

15 relations
Acid-Base EquilibriumCLCN7 geneCellular MembraneGated Ion Channel

Broader (1)

Chloride Channels

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2019
Highly Cited
2019
Lysosomal Storage and Albinism Due to Effects of a De Novo CLCN7 Variant on Lysosomal Acidification.
Highly Cited
2013
Highly Cited
2013
A model of lysosomal pH regulation
Lysosomes must maintain an acidic luminal pH to activate hydrolytic enzymes and degrade internalized macromolecules… 
Highly Cited
2011
Highly Cited
2011
ClC‐7 is a slowly voltage‐gated 2Cl−/1H+‐exchanger and requires Ostm1 for transport activity
Mutations in the ClC‐7/Ostm1 ion transporter lead to osteopetrosis and lysosomal storage disease. Its lysosomal localization… 
Highly Cited
2011
Highly Cited
2011
Degradation of Alzheimer's amyloid fibrils by microglia requires delivery of ClC-7 to lysosomes
Microglial lysosomes lack ClC-7, which leads to incomplete lysosomal acidification. In quiescent microglia, ClC-7 is targeted for… 
Highly Cited
2010
Highly Cited
2010
Lysosomal Pathology and Osteopetrosis upon Loss of H+-Driven Lysosomal Cl– Accumulation
Chloride Balancing Act The ionic composition of the cytosol and intracellular organelles must be regulated in the face of ongoing… 
2009
2009
Targeted disruption of the Cl−/HCO3− exchanger Ae2 results in osteopetrosis in mice
Osteoclasts are multinucleated bone-resorbing cells responsible for constant remodeling of bone tissue and for maintaining… 
Highly Cited
2008
Highly Cited
2008
The Cl-/H+ antiporter ClC-7 is the primary chloride permeation pathway in lysosomes
Lysosomes are the stomachs of the cell—terminal organelles on the endocytic pathway where internalized macromolecules are… 
Highly Cited
2006
Highly Cited
2006
ClC-7 requires Ostm1 as a β-subunit to support bone resorption and lysosomal function
Mutations in ClC-7, a late endosomal/lysosomal member of the CLC family of chloride channels and transporters, cause… 
2005
2005
Differential expression of the human chloride channel genes in the trabecular meshwork under stress conditions.
2004
2004
In Vitro Differentiation of CD14 Cells From Osteopetrotic Subjects: Contrasting Phenotypes With TCIRG1, CLCN7, and Attachment Defects
We studied osteoclastic differentiation from normal and osteopetrotic human CD14 cells in vitro. Defects in acid transport… 
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