Target：Glucuronidase β

Fields：Pentose and glucuronate interconversions;Ascorbate and aldarate metabolism;Glycosaminoglycan degradation;Porphyrin metabolism;Drug metabolism - other enzymes;Metabolic pathways;Biosynthesis of cofactors;Lysosome

Gene Name：GUSB

Protein Name：Beta-glucuronidase

Human Gene Id：2990

Human Swiss Prot No：P08236

Mouse Gene Id：110006

Mouse Swiss Prot No：P12265

Rat Gene Id：24434

Rat Swiss Prot No：P06760

Immunogen：The antiserum was produced against synthesized peptide derived from human GUSB. AA range:321-370

Specificity：Glucuronidase β Polyclonal Antibody detects endogenous levels of Glucuronidase β protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：IHC: 100-300.WB 1:500 - 1:2000. ELISA: 1:10000.. IF 1:50-200

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：GUSB;Beta-glucuronidase;Beta-G1

Observed Band(KD)：78kD

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],

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

Subcellular Location：Lysosome.

Expression： Colon,Fibroblast,Liver,Placenta,Plasma,