Glucuronidase β Polyclonal Antibody
- 货号：YT1920
- 应用：WB;IHC;IF;ELISA
- 种属：Human;Mouse;Rat
- 简介：
- >>Pentose and glucuronate interconversions;>>Ascorbate and aldarate metabolism;>>Glycosaminoglycan degradation;>>Porphyrin metabolism;>>Drug metabolism - other enzymes;>>Metabolic pathways;>>Biosynthesis of cofactors;>>Lysosome
- 免疫原：
- The antiserum was produced against synthesized peptide derived from human GUSB. AA range:321-370
- 特异性：
- Glucuronidase β Polyclonal Antibody detects endogenous levels of Glucuronidase β protein.
- 组成：
- Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 来源：
- Polyclonal, Rabbit,IgG
- 稀释：
- IHC: 100-300.WB 1:500 - 1:2000. ELISA: 1:10000.. IF 1:50-200
- 纯化工艺：
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
- 储存：
- -15°C to -25°C/1 year(Do not lower than -25°C)
- 其他名称：
- GUSB;Beta-glucuronidase;Beta-G1
- 背景：
- 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],
- 功能：
- 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
- 组织表达：
- Colon,Fibroblast,Liver,Placenta,Plasma,
- Immunohistochemical analysis of paraffin-embedded Human Liver. 1, Antibody was diluted at 1:100(4° overnight). 2, High-pressure and temperature EDTA, pH8.0 was used for antigen retrieval. 3,Secondary antibody was diluted at 1:200(room temperature, 30min).
- Western blot analysis of the lysates from COLO205 cells using GUSB antibody.
