Fukutin抗体;Fukutin Antibody—艾普蒂 新品

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     Western blot analysis of Fukutin expression in HeLa cell lysate.    

  
  

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     All lanes use the Antibody at 1:1K dilution for 1 hour at room temperature.    

  
  

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     All lanes use the Antibody at 1:1K dilution for 1 hour at room temperature.    

  
  

Fukutin antibody is a crucial tool in studying the molecular mechanisms underlying muscular dystrophies and related disorders. Fukutin, encoded by the *FKTN* gene, is a glycosyltransferase involved in the post-translational modification of α-dystroglycan (α-DG), a key component of the dystrophin-glycoprotein complex (DGC) that stabilizes muscle cell membranes. Mutations in *FKTN* disrupt α-DG’s glycosylation, impairing its ability to bind extracellular matrix proteins like laminin, leading to muscle degeneration and neuronal migration defects. This is associated with severe conditions such as Fukuyama congenital muscular dystrophy (FCMD), limb-girdle muscular dystrophy, and Walker-Warburg syndrome.

Fukutin-specific antibodies enable researchers to detect and quantify Fukutin protein expression in tissues or cell cultures, aiding in the diagnosis and mechanistic study of these disorders. They are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to assess protein localization, expression levels, and glycosylation status in disease models. Commercially available antibodies are typically raised against conserved epitopes in human Fukutin, often validated for cross-reactivity in murine or other experimental models. Recent studies also utilize these antibodies to evaluate experimental therapies, such as gene editing or chaperone drugs, aimed at restoring Fukutin function or α-DG glycosylation. However, challenges remain in standardizing antibody specificity due to Fukutin’s structural homology with other glycosyltransferases and low endogenous expression levels in certain tissues.