Chloride channel 7 alpha subunit (CLCN7)

Chloride channel 7 alpha subunit (CLCN7) is a voltage-gated chloride ion channel and H^+/Cl^− antiporter highly expressed on lysosomal and late endosomal membranes, where it is essential for acidification of the lysosomal lumen and maintenance of organellar pH homeostasis[2][7][9]. It operates by exchanging chloride ions for protons across the membrane, a process critical for lysosomal enzyme function, endocytosis, and bone resorption by osteoclasts[2][7][9]. Mutations in CLCN7 cause autosomal dominant osteopetrosis type II, characterized by increased bone density due to defective bone resorption[2]. Broader roles include contributions to neurodegenerative processes, highlighting its importance for general lysosomal and neuronal health[2][7][9]. CLCN7 belongs to the CLC family of chloride channels and transporters, which mediates voltage-dependent transport of chloride across membranes as either channels or exchangers[5].

Other names

H+/Cl− exchange transporter 7ClC-7CLC7chloride voltage-gated channel 7Cl− channel 7CLCN7 protein

Modulation of H^+/Cl^− exchange, inhibition or dysfunction alters lysosomal pH and disrupts acidification-dependent cellular processes