GMPPB抗体—艾普蒂

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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-2: Human cerebella tissue, Human cerebrum tissue lysates, Primary antibody: P10119(GMPPB Antibody) at dilution 1/800, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 20 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human ovarian cancer tissue using P10119(GMPPB Antibody) at dilution 1/50. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human esophagus cancer tissue using P10119(GMPPB Antibody) at dilution 1/50. (Original magnification: ×200)    

  
  

The guanosine diphosphate mannose pyrophosphorylase B (GMPPB) enzyme catalyzes the synthesis of GDP-mannose, a critical substrate for protein O- and N-linked glycosylation. Mutations in the GMPPB gene are associated with congenital muscular dystrophies (e.g., limb-girdle muscular dystrophy) and neurological disorders, often linked to defective α-dystroglycan glycosylation. GMPPB antibodies are essential tools for studying these pathologies, enabling detection of protein expression, localization, and abundance in tissues or cell models. They are widely used in techniques like Western blot, immunohistochemistry, and immunofluorescence to investigate disease mechanisms or validate GMPPB knockout models.

Most GMPPB antibodies target specific epitopes within its 360-amino acid structure, with monoclonal antibodies offering high specificity and polyclonal variants providing broader epitope recognition. Their applications extend to biomarker research, as altered GMPPB levels may correlate with disease progression. However, antibody validation remains crucial due to potential cross-reactivity with homologous proteins like GMPPA. Recent studies also explore GMPPB's role in nucleotide sugar metabolism beyond glycosylation, suggesting broader therapeutic implications. Commercially available antibodies often include validation data using knockout cell lines to ensure reliability in both research and diagnostic contexts.