HEXB抗体;HEXB Antibody—艾普蒂

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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: A172 cell lysate, Primary antibody: P13781(HEXB Antibody) at dilution 1/400, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 3 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human tonsil tissue using P13781(HEXB Antibody) at dilution 1/50. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human cervical cancer tissue using P13781(HEXB Antibody) at dilution 1/50. (Original magnification: ×200)    

  
  

HEX B antibody targets the beta subunit of β-hexosaminidase, a lysosomal enzyme critical for degrading GM2 gangliosides and other glycoconjugates. β-Hexosaminidase exists in two major isoforms: HEX A (composed of α and β subunits) and HEX B (homodimer of β subunits). HEX A deficiency causes Tay-Sachs disease, while HEX B mutations lead to Sandhoff disease, both fatal lysosomal storage disorders characterized by GM2 accumulation, neurodegeneration, and early mortality.

HEXB antibodies are essential tools in research and diagnostics. They help detect HEX B protein levels in tissues or cell cultures, assess enzyme distribution via immunohistochemistry, and validate gene-editing or therapeutic interventions in disease models. These antibodies also distinguish HEX B from HEX A in functional studies, clarifying substrate specificity and isoform interactions. Clinically, they aid in confirming Sandhoff disease diagnoses and monitoring experimental therapies, such as enzyme replacement or gene therapy.

Raised against recombinant HEX B or peptide epitopes, these antibodies require validation for specificity to avoid cross-reactivity with HEX A’s β subunit. Their applications span Western blotting, immunofluorescence, and ELISA, contributing to understanding lysosomal biology and developing targeted treatments for neurodegenerative disorders.