GUSB Recombinant Monoclonal Antibody

Code CSB-RA576809A0HU
Size US$210
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Image
  • Western Blot
    Positive WB detected in: THP-1 whole cell lysate(30µg), U-251MG whole cell lysate(30µg), A431 whole cell lysate(30µg), HeLa whole cell lysate(30µg), HepG2 whole cell lysate(30µg)
    All lanes: GUSB antibody at 1:1000
    Secondary
    Goat polyclonal to rabbit IgG at 1/40000 dilution
    Predicted band size: 75 kDa
    Observed band size: 78 kDa
    Exposure time:3min
  • IHC image of CSB-RA576809A0HU diluted at 1:100 and staining in paraffin-embedded human colorectal cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
  • IHC image of CSB-RA576809A0HU diluted at 1:100 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
  • Immunofluorescence staining of MCF-7 cell with CSB-RA576809A0HU at 1:50, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • Overlay Peak curve showing 786-O cells stained with CSB-RA576809A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for 10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
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Product Details

Uniprot No.
Target Names
Alternative Names
Beta-glucuronidase (EC 3.2.1.31) (Beta-G1), GUSB
Species Reactivity
Human
Immunogen
A synthesized peptide from human GUSB protein
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Clonality
Monoclonal
Isotype
Rabbit IgG
Clone No.
11D12
Purification Method
Affinity-chromatography
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
Form
Liquid
Tested Applications
ELISA, WB, IHC, IF, FC
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
IHC 1:50-1:200
IF 1:50-1:200
FC 1:50-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Description

Beta-glucuronidase, encoded by the GUSB gene, is a lysosomal enzyme essential for the degradation of glycosaminoglycans within the cell. Deficiency in this enzyme leads to mucopolysaccharidosis type VII, making it a significant target for researchers investigating lysosomal storage disorders, glycosaminoglycan metabolism, and broader aspects of cellular degradation pathways. GUSB also serves as a commonly used housekeeping gene in expression studies, adding to its utility across diverse research contexts.

This recombinant monoclonal antibody, clone 11D12, offers the consistency and reproducibility that demanding experimental workflows require. Because it is produced using recombinant technology with a defined sequence, you can expect reliable lot-to-lot performance, eliminating the variability often encountered with traditional hybridoma-derived antibodies. This consistency is particularly valuable when establishing long-term studies or comparing results across experiments.

The antibody has been validated across multiple applications, providing flexibility for your research needs. In western blot analysis, it detects GUSB in a range of human cell lines including THP-1, U-251MG, A431, HeLa, and HepG2, with an observed band at approximately 78 kDa. The slight shift from the predicted 75 kDa molecular weight likely reflects post-translational modifications such as glycosylation, which is characteristic of lysosomal enzymes. Immunohistochemistry validation demonstrates clear staining in human colorectal cancer and liver tissue sections, while immunofluorescence studies in MCF-7 cells and flow cytometry analysis in 786-O cells confirm its utility for cellular localization and quantitative studies.

This antibody is well-suited for researchers exploring lysosomal biology, cancer metabolism, or glycosaminoglycan-related pathways requiring a dependable detection tool for human GUSB.

Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function
Plays an important role in the degradation of dermatan and keratan sulfates.
Gene References into Functions
  1. Results show that GUS mRNA changes in blood are significantly correlated with gait step length variability in premutation (PM) females patients with fragile X tremor ataxia syndrome . PMID: 27387142
  2. We produced GUS from a CHO cell line grown in suspension in a 15 L perfused bioreactor and developed a three step purification procedure that yields approximately 99% pure enzyme with a recovery of more than 40%. PMID: 28734840
  3. Selective and amplified bG expression together with the prodrug DOXGA3 had an increased antitumor effect, showing great potential for prostate cancer therapy. PMID: 26648021
  4. the use of in silico approaches provided a useful understanding of the effect of single point mutations on the structure-function relationship of GUSBp PMID: 26415878
  5. the efficacy of a helper-dependent (HD) canine adenovirus (CAV-2) vector harboring a human GUSB expression cassette (HD-RIGIE) in the MPS VII dog brain was tested PMID: 24343103
  6. This assessment demonstrated that although butyrate dramatically increased beta-glucuronidase production in bioreactors, it adversely impacted the mannose-6-phosphorylation of this lysosomal storage diseasestherapeutic enzyme PMID: 24033810
  7. expression of CES2, UGTA1A1, and GUSB varies in colorectal pathology tissues and that the expression of CES2 is somewhat related to tumor staging. PMID: 24195516
  8. GUSB and ATP2B4 have been validated as a reliable gene combination for Cystic Fibrosis Transmembrane Conductance Regulator gene qPCR data normalization. PMID: 22525089
  9. Liver disease affects the expression of common HKGs; beta-glucuronidase and splicing factor arginine/serine-rich 4 are the most stable HKGs for studies of gene expression in HCV-infected human liver. PMID: 21073651
  10. beta-glucuronidase mutations are associated with mucopolysaccharidosis type VII PMID: 12522561
  11. Recombinant adeno-associated virus encoding human GUSB injected into the vitreous humor of young adult MPS VII mice increased GUSB activity and reduced lysosomal distension in regions of the thalamus and tectum PMID: 12716937
  12. beta glucuronidase present in the synovial fluid of rheumatoid arthritis patients, may contribute to the depletion of glycosaminoglycans from cartilage allowing invasion of synovial cells. PMID: 12905469
  13. Over-expression of beta-glucuronidase was related to the degree of cancer differentiation, but not to lymph node metastasis. PMID: 14612292
  14. These results indicate that beta-glucuronidase transport into brain parenchyma in early postnatal life is mediated by the mannose 6-phosphate/insulin-like growth factor II receptor. PMID: 15314220
  15. The genes GUS and PMM1 are recommended for normalization purposes in gene expression studies of liver tissue from patients with chronic hepatitis. PMID: 18591914
  16. Study summarized information on the 49 unique, disease-causing mutations determined so far in the GUS gene. PMID: 19224584

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Involvement in disease
Mucopolysaccharidosis 7 (MPS7)
Subcellular Location
Lysosome.
Protein Families
Glycosyl hydrolase 2 family
Database Links

HGNC: 4696

OMIM: 253220

KEGG: hsa:2990

STRING: 9606.ENSP00000302728

UniGene: Hs.255230

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