GBE1抗体—艾普蒂

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     The image is immunohistochemistry of paraffin-embedded Human gastric cancer tissue using P10455(GBE1 Antibody) at dilution 1/80. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human colorectal cancer tissue using P10455(GBE1 Antibody) at dilution 1/80. (Original magnification: ×200)    

  
  

The GBE1 antibody targets glycogen branching enzyme 1 (GBE1), a protein critical for glycogen metabolism. GBE1 catalyzes the formation of α-1.6-glycosidic branches during glycogen synthesis, enabling efficient glucose storage and mobilization. Mutations in the GBE1 gene are linked to glycogen storage disease type IV (GSD-IV, Andersen disease), a rare autosomal recessive disorder characterized by abnormal glycogen accumulation in tissues, leading to liver cirrhosis, neuromuscular dysfunction, and cardiomyopathy. The severe infantile form is often fatal, while milder adult-onset variants may present with myopathy or cardiomyopathy.

GBE1 antibodies are essential tools for studying GSD-IV pathogenesis, diagnosing genetic variants, and exploring therapeutic strategies. These antibodies are widely used in techniques like Western blotting, immunohistochemistry (IHC), and immunofluorescence (IF) to detect GBE1 expression levels, localization, and structural abnormalities in tissues or cell models. Commercially available GBE1 antibodies are typically raised against specific epitopes, with polyclonal antibodies offering broad detection sensitivity and monoclonal antibodies providing high specificity. Validation often includes testing in GBE1-deficient samples to confirm target specificity. Researchers also utilize recombinant GBE1 proteins expressed in systems like HEK293 or E. coli for antibody validation and functional assays.