Molecular weight (DA)
78kD
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.
Dilution rate
IHC-p: 100-300.WB: 1/500 - 1/2000. ELISA: 1/10000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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
Protein name
Beta-glucuronidase
Abbreviation
Glucuronidase β
Other name
GUSB; Beta-glucuronidase; Beta-G1
Fields
>>Pentose and glucuronate interconversions;>>Ascorbate and aldarate metabolism;>>Glycosaminoglycan degradation;>>Porphyrin metabolism;>>Drug metabolism - other enzymes;>>Metabolic pathways;>>Biosynthesis of cofactors;>>Lysosome
Human protein sequence Database
P08236
Mouse protein sequence database
P12265
Rat protein sequence database
P06760
Cellular localization
Lysosome.
Tissue expression
Colon,Fibroblast,Liver,Placenta,Plasma,
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