Product nameAnti-beta glucuronidase (GUSB) antibody 
See all beta glucuronidase (GUSB) primary antibodies
DescriptionMouse monoclonal  to beta glucuronidase (GUSB)
SpecificityThis antibody reacts with an non-active center epitope on human placental and liver Beta Glucuronidase. The antibody apparently reacts with the native enzyme only.
Tested applicationsSuitable for: ELISA, IHC-Fr, WB, Othermore details
Species reactivityReacts with: Human
Does not react with: Rat
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.1% Sodium azide
PurityTissue culture supernatant
Our Abpromise guarantee covers the use of ab8478 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
WB: 1/10. The beta-glucuronidase is in its native form a tetramer of MW 280 kDa. Furthermore it can be glycosylated.
Not yet tested in other applications.
Optimal dilutions/concentrations should be determined by the end user.
FunctionPlays an important role in the degradation of dermatan and keratan sulfates.
Involvement in diseaseDefects 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.
Note=Mucopolysaccharidosis type 7 is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.
Sequence similaritiesBelongs to the glycosyl hydrolase 2 family.
modificationsN-linked glycosylated with 3 to 4 oligosaccharide chains.
- Information by UniProt
- Ac2-223 antibody
- asd antibody
- Beta G1 antibody
ab8478 has not yet been referenced specifically in any publications.