Galactosidasealpha抗体;Galactosidasealpha Antibody—艾普蒂 新品

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     Western blot analysis of Galactosidase alpha expression in MCF-7 cell lysate.    

  
  

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

  
  

Galactosidase alpha (α-galactosidase A, GLA) antibodies are immunological tools specifically designed to detect and study the α-galactosidase A enzyme, a lysosomal hydrolase critical for glycolipid metabolism. This enzyme catalyzes the hydrolysis of globotriaosylceramide (Gb3) and other glycosphingolipids, and its deficiency leads to Fabry disease, a rare X-linked lysosomal storage disorder. Antibodies against GLA are widely used in research and diagnostics to assess enzyme expression, localization, and activity in tissues or cell cultures, aiding in the understanding of Fabry disease pathogenesis.

These antibodies are employed in techniques like ELISA, Western blotting, and immunohistochemistry to quantify protein levels, evaluate mutations, or monitor therapeutic efficacy in enzyme replacement therapy (ERT) or gene therapy. Commercially available monoclonal or polyclonal GLA antibodies target specific epitopes, enabling precise detection of both endogenous and recombinant forms of the enzyme. Additionally, they help identify cross-reactive immunological material (CRIM) in patients, guiding personalized treatment strategies. In clinical diagnostics, GLA antibodies assist in confirming Fabry disease by detecting reduced enzyme activity or abnormal protein accumulation. Their role extends to developing novel therapies, including substrate reduction therapy and chaperone-mediated approaches. As research advances, GLA antibodies remain pivotal in bridging molecular insights with therapeutic innovations for lysosomal disorders.