Boster Immunoleader

antibody

Anti-beta glucuronidase(GUSB) Antibody

PA1916

100 ug/vial

200 USD

polyclonal

rabbit

Anti-beta glucuronidase(GUSB) Antibody

PA1916

Polyclonal

Rabbit IgG polyclonal antibody for Beta-glucuronidase(GUSB) detection. Tested with WB in Human.

200 USD

Rabbit IgG

100 ug/vial

Lyophilized

No cross reactivity with other proteins.

Reacts with: human

WB

Concentration: 0.1-0.5μg/ml; Tested Species: Human

WB: The detection limit for GUSB is approximately 2.5ng/lane under reducing conditions. Tested Species: In-house tested species with positive results. Predicted Species: Species predicted to be fit for the product based on sequence similarities. Other applications have not been tested. Optimal dilutions should be determined by end users.

A synthetic peptide corresponding to a sequence at the N-terminus of human beta glucuronidase(GUSB)(50-70aa DFSDNRRRGFEEQWYRRPLWE).

Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.

Immunogen affinity purified.

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

At -20˚C for one year. After reconstitution, at 4˚C for one month. It can also be aliquotted and stored frozen at -20˚C for a longer time. Avoid repeated freezing and thawing.

Boster provides a series of assays reacted with primary antibodies. Antibody can be supported by chemiluminescence kit EK1002 in WB.

GUSB(Beta-Glucuronidase) is an enzyme that in humans is encoded by the GUSB gene. The GUSB gene encodes beta-glucuronidase, a lysosomal hydrolase involved in the stepwise degradation of glucuronic acid-containing glycosaminoglycans. It is a tetrameric glycoprotein composed of identical subunits. The GUSB gene is mutated in mucopolysaccharidosis type VII. Miller et al.(1990) reported that the GUSB gene is 21 kb long and contains 12 exons. The GUSB gene is mapped on 7q11.21. Beta-glucuronic acid acts naturally to cleave glucuronides from conjugated bilirubin to produce unconjugated bilirubin, which is then absorbed into the bloodstream.Kreamer et al.(2001) postulated that the beta-glucuronidase inhibitor L-aspartic acid can thus block enterohepatic circulation of bilirubin. Tomatsu et al.(2002) stated that more than 45 different mutations in the GUSB gene had been identified in human patients with MPS VII, approximately 90% of which were point mutations.

1. Kreamer, B. L., Siegel, F. L., Gourley, G. R. A novel inhibitor of beta-glucuronidase: L-aspartic acid. Pediat. Res. 50: 460-466, 2001. 2. Miller, R. D., Hoffmann, J. W., Powell, P. P., Kyle, J. W., Shipley, J. M., Bachinsky, D. R., Sly, W. S. Cloning and characterization of the human beta-glucuronidase gene. Genomics 7: 280-283, 1990. 3. Tomatsu, S., Orii, K. O., Vogler, C., Grubb, J. H., Snella, E. M., Gutierrez, M. A., Dieter, T., Sukegawa, K., Orii, T., Kondo, N., Sly, W. S. Missense models [Gus(tm(E536A)Sly), Gus(tm(E536Q)Sly), and Gus(tm(L175F)Sly)] of murine mucopolysaccharidosis type VII produced by targeted mutagenesis. Proc. Nat. Acad. Sci. 99: 14982-14987, 2002.

GUSB

Beta-glucuronidase

glucuronidase, beta

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P08236