GUSB抗体—艾普蒂

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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: K562 cell lysate, Primary antibody: P10208(GUSB Antibody) at dilution 1/800, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 5 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human cervical cancer tissue using P10208(GUSB Antibody) at dilution 1/50. (Original magnification: ×200)    

  
  

**Background of GUSB Antibodies**

Beta-glucuronidase (GUSB) is a lysosomal enzyme encoded by the *GUSB* gene, responsible for hydrolyzing glycosaminoglycans (GAGs), such as dermatan sulfate and heparan sulfate, by cleaving terminal glucuronic acid residues. Deficiencies in GUSB activity lead to mucopolysaccharidosis type VII (MPS VII, Sly syndrome), a rare lysosomal storage disorder characterized by GAG accumulation, resulting in multisystemic complications.

GUSB antibodies are immunodetection tools targeting this enzyme, widely used in research and diagnostics. Polyclonal or monoclonal antibodies are developed using purified GUSB protein or peptide antigens. These antibodies enable the detection of GUSB expression levels via techniques like Western blotting, immunohistochemistry (IHC), immunofluorescence (IF), or ELISA. They are critical for studying GUSB localization, expression patterns, and enzymatic activity in cellular and tissue models.

In research, GUSB antibodies aid in investigating lysosomal function, metabolic diseases, and GUSB-related pathways. They are also employed to validate gene-editing outcomes (e.g., CRISPR/Cas9) or recombinant GUSB therapies in preclinical models of MPS VII. Additionally, GUSB is often used as a reporter gene or internal control in molecular biology due to its stability and ease of detection. Commercial GUSB antibodies are rigorously validated for specificity, often using knockout cell lines or tissue samples.

Overall, GUSB antibodies serve as essential reagents for understanding lysosomal biology, disease mechanisms, and therapeutic development for metabolic disorders.