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Products Polyclonal Anti-GUSB
| Catalogue number: | PA1916 |
| Price: | $200.00 |
| Reactivities: | Human |
| Applications: | Western Blot |
| Size: | 100ug/vial |
| Gene: | GUSB |
| Swiss prot: | P08236 |
| Form: | Lyophilized |
| Format: | Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. |
| Storage temp: | At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time.Avoid repeated freezing and thawing. |
| Scientific background: | 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. |
| References: | 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. |
| Additional info: | A synthetic peptide corresponding to a sequence at the N-terminal of human GUSB. |
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