CLN5抗体;CLN5 Antibody—艾普蒂

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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: 231 cell lysate, Primary antibody: P13095(CLN5 Antibody) at dilution 1/200, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 10 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P13095(CLN5 Antibody) at dilution 1/20. (Original magnification: ×200)    

  
  

CLN5 antibodies are immunological tools used to study the CLN5 protein, which is associated with neuronal ceroid lipofuscinosis type 5 (CLN5 disease), a form of Batten disease. CLN5 disease is a rare, inherited neurodegenerative disorder classified under the neuronal ceroid lipofuscinoses (NCLs), a group of lysosomal storage disorders. Mutations in the *CLN5* gene lead to defective lysosomal function, resulting in the accumulation of lipopigments in neurons and other cells, progressive neurodegeneration, and symptoms such as vision loss, motor decline, seizures, and cognitive impairment.

The CLN5 protein, predominantly expressed in lysosomes and endosomes, is implicated in lipid metabolism, membrane trafficking, and autophagy, though its precise molecular function remains under investigation. Antibodies targeting CLN5 are essential for detecting its expression, localization, and interaction partners in cellular and animal models. They enable researchers to study protein distribution in tissues, assess expression levels in disease states, and validate experimental models (e.g., CLN5-deficient mice or zebrafish).

CLN5 antibodies also support diagnostic workflows, helping distinguish CLN5-related pathology from other NCL subtypes. Additionally, they play a role in evaluating therapeutic strategies, such as gene therapy or enzyme replacement, by monitoring CLN5 restoration or downstream lysosomal activity. As CLN5 research advances, these antibodies remain critical for unraveling disease mechanisms and developing targeted interventions.