ITT1920
ITT1920
- Catalog: ITT1920
- Gene/Protein: GUSB
- Product Description: Immunotag™ Glucuronidase β Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
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| Target Protein | Glucuronidase β |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,IHC-p,ELISA |
| Recommended Dilution | IHC-p: 100-300.Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications. |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse,Rat |
| Host Species | Rabbit |
| Immunogen | Synthesized peptide derived from Glucuronidase β, at AA range: 290-370 |
| Specificity | Glucuronidase β Polyclonal Antibody detects endogenous levels of Glucuronidase β protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | GUSB |
| Accession No. | P08236 P12265 P06760 |
| Alternate Names | GUSB; Beta-glucuronidase; Beta-G1 |
| Description | glucuronidase beta(GUSB) Homo sapiens This gene encodes a hydrolase that degrades glycosaminoglycans, including heparan sulfate, dermatan sulfate, and chondroitin-4,6-sulfate. The enzyme forms a homotetramer that is localized to the lysosome. Mutations in this gene result in mucopolysaccharidosis type VII. Alternative splicing results in multiple transcript variants. There are many pseudogenes of this locus in the human genome.[provided by RefSeq, May 2014], |
| Cell Pathway/ Category | Pentose and glucuronate interconversions,Starch and sucrose metabolism,Glycosaminoglycan degradation,Porphyrin and chlorophyll metabolism,Drug metabolism,Lysosome, |
| Protein Expression | Colon,Fibroblast,Liver,Placenta,Plasma, |
| Subcellular Localization | extracellular space,membrane,lysosomal lumen,intracellular membrane-bounded organelle,extracellular exosome, |
| Protein Function | catalytic activity:A beta-D-glucuronoside + H(2)O = D-glucuronate + an alcohol.,disease:Defects in GUSB are the cause of mucopolysaccharidosis type 7 (MPS7) [MIM:253220]; 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.,disease:Mucopolysaccharidosis type 7 is associated with non-immune hydrops fetalis [MIM:236750]. Hydrops fetalis is a generalized edema of the fetus with fluid accumulation in the body cavities.,enzyme regulation:Inhibited by L-aspartic acid.,function:Plays an important role in the degradation of dermatan and keratan sulfates.,PTM:N-linked glycosylated with 3 to 4 oligosaccharide chains.,similarity:Belongs to the glycosyl hydrolase 2 family.,subunit:Homotetramer., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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