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Merck
CN

SAB4200336

Sigma-Aldrich

Anti-GUSB (C-terminal) antibody produced in rabbit

~1.0 mg/mL, affinity isolated antibody

别名:

Anti-β-D-Glucuronidase, Anti-β-Glucuronidase, Anti-β-g1, Anti-MPS7

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关于此项目

UNSPSC代码:
12352203
NACRES:
NA.41
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生物来源

rabbit

偶联物

unconjugated

抗体形式

affinity isolated antibody

抗体产品类型

primary antibodies

克隆

polyclonal

表单

buffered aqueous solution

分子量

antigen 78 kDa

种属反应性

rat, human

浓度

~1.0 mg/mL

技术

immunohistochemistry: 10-20 μg/mL using biotin / ExtrAvidin®-Peroxidase staining of heat-retrieved formalin-fixed, paraffin-embedded rat liver sections
western blot: 2-4 μg/mL using whole extracts of human HepG2 cells

UniProt登记号

运输

dry ice

储存温度

−20°C

靶向翻译后修饰

unmodified

基因信息

human ... GUSB(2990)
mouse ... Gusb(110006)

一般描述

Glucuronidase β (GUSB), an exoglycosidase, is a member of the glycosyl hydrolase 2 family. The GUSB protein has a homotetrameric structure that is found in the lysosome. It contains three structural domains, such as a jelly roll barrel, an immunoglobulin constant domain and a triosephosphate isomerase (TIM) barrel respectively. The gene GUSB is mapped to the human chromosome location 7q11.21.

免疫原

a synthetic peptide corresponding to a sequence at the C-terminal region of human GUSB, conjugated to KLH. The corresponding sequence is identical in mouse and differs by a single amino acid in rat.

应用

Anti-GUSB (C-terminal) antibody produced in rabbit may be used in immunoblotting and immunohistochemistry.

生化/生理作用

Anti-GUSB (C-terminal) recognizes human and rat GUSB.
Glucuronidase β (GUSB) is a hydrolase that degrades glycosaminoglycans (GAGs), such as heparan sulfate, dermatan sulfate and chondroitin-4,6-sulfate. In the absence of GUS, only partial degradation of GAGs takes place. This leads to the accumulation of GAGs in the lysosomes of many tissues, leading to cellular and organ dysfunction. Mutations in this gene lead to mucopolysaccharidosis type VII (Sly syndrome). Several pseudogenes of this locus are present in the human genome.

外形

Solution in 0.01 M phosphate buffered saline pH 7.4, containing 15 mM sodium azide.

制备说明

For continuous use, store at 2-8°C for up to one month. For extended storage freeze in working aliquots. Repeated freezing and thawing is not recommended. If slight turbidity occurs upon prolonged storage, clarify the solution by centrifugation before use. Working dilution samples should be discarded if not used within 12 hours.

法律信息

ExtrAvidin is a registered trademark of Merck KGaA, Darmstadt, Germany

免责声明

Unless otherwise stated in our catalog, our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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储存分类代码

10 - Combustible liquids

闪点(°F)

Not applicable

闪点(°C)

Not applicable

法规信息

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分析证书(COA)

Lot/Batch Number

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访问文档库

Mutations and polymorphisms in GUSB gene in mucopolysaccharidosis VII (Sly Syndrome)
Tomatsu S, et al.
Human Mutation, 30(4), 511 -5519 (2009)
The structural basis of substrate recognition in an exo-beta-D-glucosaminidase involved in chitosan hydrolysis
van Bueren AL, et al.
Journal of molecular biology, 385(1), 131 -1139 (2009)
Structure of human beta-glucuronidase reveals candidate lysosomal targeting and active-site motifs
Jain S, et al.
Nature Structural Biology, 3(4), 375 -3381 (1996)

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