CEP290抗体—艾普蒂

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     The image is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using P09484(CEP290 Antibody) at dilution 1/25. (Original magnification: ×200)    

  
  

The CEP290 antibody targets the centrosomal protein 290 (CEP290), a large, multidomain protein critical for ciliogenesis, cell cycle regulation, and centrosomal function. CEP290 localizes to the centrosome and cilium transition zone, where it facilitates ciliary membrane biogenesis, protein trafficking, and structural integrity. Mutations in the CEP290 gene are linked to severe ciliopathies, including Leber congenital amaurosis (LCA), Joubert syndrome, and Meckel-Gruber syndrome, often causing retinal degeneration, renal abnormalities, and neurodevelopmental defects.

As a research tool, CEP290 antibodies are widely used in immunofluorescence, Western blotting, and immunohistochemistry to study ciliary architecture, centrosome dynamics, and disease mechanisms. These antibodies help identify CEP290 expression patterns, subcellular localization, and interactions with proteins like NPHP, RPGR, and BBS proteins, shedding light on ciliary signaling pathways. Their applications extend to diagnosing ciliopathies and validating cellular or animal models of CEP290 dysfunction.

Given CEP290's role in maintaining sensory cilia (e.g., photoreceptors), antibodies also aid in exploring therapeutic strategies, such as gene editing or small-molecule interventions. Cross-reactivity with orthologs in model organisms (e.g., mice, zebrafish) enables translational studies. However, challenges remain due to CEP290's size (~290 kDa) and complex domain structure, requiring careful validation of antibody specificity to avoid off-target signals in experimental assays.