bsm-54726R [Primary Antibody]
Beta glucuronidase Monoclonal Antibody
www.biossusa.com
[email protected]
800.501.7654 [DOMESTIC]
+1.781.569.5821 [INTERNATIONAL]
DATASHEET

Host: Rabbit

Target Protein: Beta glucuronidase

Clonality: Monoclonal

Isotype: IgG

Entrez Gene: 2990

Swiss Prot: P08236

Source: Synthetic Peptide within Human beta glucuronidase

Purification: Purified by Protein A.

Storage Buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at -20°C for 12 months.

Background:

Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7) ; also known as Sly syndrome. MPS7 is an autosomal recessive lysosomal storage disease characterized by inability to degrade glucuronic acid-containing glycosaminoglycans. The phenotype is highly variable, ranging from severe lethal hydrops fetalis to mild forms with survival into adulthood. Most patients with the intermediate phenotype show hepatomegaly, skeletal anomalies, coarse facies, and variable degrees of mental impairment. Mucopolysaccharidosis type 7 is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.

Size: 100ul

Concentration: 1ug/ul

Predicted Molecular Weight: 75


Cross Reactive Species: Human

For research use only. Not intended for diagnostic or therapeutic use.

VALIDATION IMAGES

Paraformaldehyde-fixed, paraffin embedded Human prostate cancer; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with Beta glucuronidase Monoclonal Antibody, Unconjugated (bsm-54726R) at 1:200 for 30 minutes at room temperature, DAB staining.


Paraformaldehyde-fixed, paraffin embedded Human kidney; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with Beta glucuronidase Monoclonal Antibody, Unconjugated (bsm-54726R) at 1:200 for 30 minutes at room temperature, DAB staining.


THP-1 cells were fixed,permeabilized and incubated in 5% BSA blocking buffer for 30 min at room temperature. Cells were then stained with Beta glucuronidase Monoclonal Antibody(bsm-54726R)at 1:50 dilution in blocking buffer and incubated for 30 min at room temperature, washed twice with 2%BSA in PBS, followed by secondary antibody incubation for 40 min at room temperature. Acquisitions of 20,000 events were performed. Cells stained with primary antibody (red).