Human beta -Glucuronidase (EC 3.2.1.31) encoded by the GUSB gene is a lysosomal hydrolase involved in the stepwise degradation of glucuronic acid-containing glycosaminoglycans that include heparan sulfate, chondroitin sulfate and hyaluronan (1). The enzyme is only active on the glucuronic acid of the non-reducing end. The native protein has been reported as a tetrameric glycoprotein composed of identical subunits (1, 2). Mutations in the GUSB gene are linked to mucopolysaccharidosis type VII (3). Accumulation of partially degraded glycosaminoglycans, with glucuronic acid residues at the non-reducing termini, are usually found in the lysosomes of patients with the disease (3). It has also been reported that this enzyme may contribute to the depletion of chondroitin from cartilage and thereby facilitate the damage of joints in rheumatoid arthritis (4).
Human beta-Glucuronidase/GUSB Alexa Fluor™ Plus 555‑conjugated Antibody
R&D Systems | Catalog # AF6144AFP555
Key Product Details
Species Reactivity
Applications
Label
Antibody Source
Product Specifications
Specificity
Clonality
Host
Isotype
Applications
Western Blot
Formulation, Preparation, and Storage
Formulation
Shipping
Stability & Storage
Background: beta-Glucuronidase/GUSB
References
- Shipley, J.M. et al. (1993) Am. J. Hum. Genet. 52:517.
- Oshima, A, et al. (1987) Proc. Natl. Acad. Sci. USA 84:685.
- Bell, C.E. Jr. et al. (1977) J. Clin. Invest. 59:97.
- Ortutay, Z. et al. (2003) Arthritis Rheum. 48:2163.
Alternate Names
Entrez Gene IDs
Gene Symbol
UniProt
Additional beta-Glucuronidase/GUSB Products
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Product Specific Notices
This product is provided under an intellectual property license from Life Technologies Corporation. The transfer of this product is conditioned on the buyer using the purchased product solely in research conducted by the buyer, excluding contract research or any fee for service research, and the buyer must not (1) use this product or its components for (a) diagnostic, therapeutic or prophylactic purposes; (b) testing, analysis or screening services, or information in return for compensation on a per-test basis; or (c) manufacturing or quality assurance or quality control, and/or (2) sell or transfer this product or its components for resale, whether or not resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.
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Protocols
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- Cellular Response to Hypoxia Protocols
- R&D Systems Quality Control Western Blot Protocol
- Troubleshooting Guide: Western Blot Figures
- Western Blot Conditions
- Western Blot Protocol
- Western Blot Protocol for Cell Lysates
- Western Blot Troubleshooting
- Western Blot Troubleshooting Guide
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