SUBCELLULAR LOCALIZATION
| intracellular
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| intracellular,cytoplasm,organelle,membrane
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| intracellular,cytoplasm,organelle,endoplasmic reticulum
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| intracellular,cytoplasm,organelle,endosome
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| intracellular,cytoplasm,organelle,lysosome
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| intracellular,cytoplasm,cytosolic,vesicle
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| text
| co-localized with OSTM1 in late endosomal/lysosomal Cl(-) channel, inserted in the ruffled border of bone-resorbing osteoclasts, by exocytic fusion with the H(+)-ATPase |
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is synthesized by microglia but it is mistargeted and appears to be degraded by an endoplasmic reticulum-associated degradation pathway  |
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located in late endosomes and lysosomes |
| basic FUNCTION
| chloride voltage-gated channel, providing the chloride conductance requested for an efficient proton pumping by the H+-ATPase of the osteoclast ruffled membrane |
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chloride channel of late endosomes and lysosomes, playing a pivotal role in acidifying the extracellular lysosomal membrane between osteoclast and bone |
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voltage-gated chloride channel involved in the regulation of cell volume, membrane potential stabilization, signal transduction and transepithelial transport |
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Cl-/H+ antiporter, that it constitutes the major Cl- permeability of lysosomes, and that it is important in lysosomal acidification |
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CLCN7 functions as an antiporter |
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implied in the pathogenesis of lysosomal storage disease and osteopetrosis |
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CLCN7 associates with another protein, OSTM1, which plays an important role in its correct lysosomal targeting |
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is essential for osteoclasts to resorb craniofacial bones to enable tooth eruption and root development |
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CLCN7 may affect tooth development by directly targeting tooth cells, and regulate tooth eruption through dental follicle cells (DFCs) mediated osteoclast pathway |
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regulates the pattern and early development of craniofacial bone and tooth |
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critical role of the CLCN7 chloride/proton antiporter in maintaining lysosomal pH within a very narrow range |
