CLCN7抗体—艾普蒂

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     Gel: 6%SDS-PAGE, Lysate: 40 μg, Lane 1-3: HepG2, A549, LOVO cell lysates, Primary antibody: P09519(CLCN7 Antibody) at dilution 1/500, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 2 minutes    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using P09519(CLCN7 Antibody) at dilution 1/100. (Original magnification: ×200)    

  
  

The CLCN7 antibody targets the CLCN7 protein, a member of the CLC family of voltage-gated chloride channels/transporters. CLCN7 encodes the ClC-7 chloride channel, which forms a dimeric structure with two independent ion-conducting pores. Primarily localized in lysosomes and the ruffled border of osteoclasts, ClC-7 plays a critical role in lysosomal pH regulation and bone resorption by facilitating chloride ion transport. Mutations in the CLCN7 gene are linked to osteopetrosis, a rare genetic disorder characterized by increased bone density due to impaired osteoclast activity. Specifically, autosomal recessive osteopetrosis (ARO) and autosomal dominant osteopetrosis type 2 (ADO2) are associated with loss-of-function or dominant-negative mutations, respectively. Additionally, ClC-7 dysfunction may contribute to lysosomal storage disorders and neurodegenerative phenotypes.

CLCN7 antibodies are widely used in research to study protein expression, subcellular localization, and functional alterations in disease models. They enable detection of ClC-7 in tissues like bone, brain, and kidney via techniques such as Western blotting, immunohistochemistry, and immunofluorescence. These antibodies also aid in exploring therapeutic strategies targeting osteoclast activity or lysosomal function. However, challenges remain in distinguishing pathogenic mutations from benign variants and understanding tissue-specific regulatory mechanisms. Ongoing studies aim to clarify ClC-7's interactions with accessory proteins (e.g., OSTM1) and its role beyond ion transport, such as in cellular autophagy and signaling pathways.