Key features and details
- Mouse monoclonal  to beta glucuronidase (GUSB)
- Suitable for: ELISA, IHC-Fr, WB, Other
- Reacts with: Human
- Isotype: IgG1
製品名Anti-beta glucuronidase (GUSB) antibody 
beta glucuronidase (GUSB) 一次抗体 製品一覧
製品の詳細Mouse monoclonal  to beta glucuronidase (GUSB)
特異性This antibody reacts with an non-active center epitope on human placental and liver Beta Glucuronidase. The antibody apparently reacts with the native enzyme only.
アプリケーション適用あり: ELISA, IHC-Fr, WB, Othermore details
保存方法Shipped 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.
バッファーPreservative: 0.1% Sodium azide
Concentration information loading...
精製度Tissue 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.
機能Plays an important role in the degradation of dermatan and keratan sulfates.
関連疾患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.
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.
配列類似性Belongs to the glycosyl hydrolase 2 family.
翻訳後修飾N-linked glycosylated with 3 to 4 oligosaccharide chains.
- Information by UniProt
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