Price range: $268.00 through $328.00
Background:
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],
| Target | Glucuronidase β |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, ELISA |
| MW(Calculated) | |
| MW(Observed) | 78kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Modification | |
| Recommended Dilution Ratio | IHC: 100-300; WB 1:500-1:2000; ELISA 1:10000; IF 1:50-200 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#YS0004) |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Source | |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Purity | |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | The antiserum was produced against synthesized peptide derived from human GUSB. AA range:321-370 |
| Sequence | |
| Specificity | Glucuronidase β Polyclonal Antibody detects endogenous levels of Glucuronidase β protein. |
| Gene Name | GUSB |
| Protein Name | Beta-glucuronidase |
| Other Name | GUSB; / Beta-glucuronidase; / Beta-G1 |
| Species | Human |
| Gene ID-1 | 2990 |
| Uniprot | P08236, |
| Species.1 | Mouse |
| Gene ID-2 | 110006 |
| Uniprot.1 | P12265, |
| Species.2 | Rat |
| Gene ID-3 | 24434 |
| Uniprot.2 | P06760 |
| Organism-4 | |
| Gene ID-4 | |
| SwissProt-4 | |
| Background | 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], |
| Cellular Localization | Lysosome. |
| Tissue Expression | Colon,Fibroblast,Liver,Placenta,Plasma, |
| Signaling_pathway | Cellular Processes >> Transport and catabolism >> Lysosome |
| Research Areas | >>Pentose and glucuronate interconversions; / >>Ascorbate and aldarate metabolism; / >>Glycosaminoglycan degradation; / >>Porphyrin metabolism; / >>Drug metabolism - other enzymes; / >>Metabolic pathways; / >>Biosynthesis of cofactors; / >>Lysosome |
| 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., |
| RRID |
