Key features and details
- Rabbit polyclonal to beta glucuronidase (GUSB)
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Product nameAnti-beta glucuronidase (GUSB) antibody
See all beta glucuronidase (GUSB) primary antibodies
DescriptionRabbit polyclonal to beta glucuronidase (GUSB)
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat
Synthetic peptide derived from internal amino acids of Human beta glucuronidase (GUSB).
- COLO205 cell lysate.
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Storage instructionsShipped at 4°C. Store at -20ºC.
Storage bufferpH: 7.40
Preservative: 0.02% Sodium azide
Constituents: 49% PBS, 50% Glycerol (glycerin, glycerine), 0.88% Sodium chloride
PBS is without Mg2+ and Ca2+
Concentration information loading...
PurityImmunogen affinity purified
Purification notesab136860 was affinity-purified from rabbit antiserum by immunogenic peptide affinity-chromatography.
Our Abpromise guarantee covers the use of ab136860 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/1000. Predicted molecular weight: 75 kDa.|
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
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ab136860 has been referenced in 1 publication.
- Bhamidimarri PM et al. Mannose 6-phosphate-dependent lysosomal enzyme targeting in hydra: a biochemical, immunological and structural elucidation. FEBS Lett 592:1366-1377 (2018). PubMed: 29537487