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Key Documents

SAB4200336

Sigma-Aldrich

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

~1.0 mg/mL, affinity isolated antibody

Synonym(s):

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

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

biological source

rabbit

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

antigen 78 kDa

species reactivity

rat, human

concentration

~1.0 mg/mL

technique(s)

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 accession no.

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

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

General description

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.

Specificity

Anti-GUSB (C-terminal) recognizes human and rat GUSB.

Immunogen

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.

Application

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

Biochem/physiol Actions

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.

Physical form

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

Storage and Stability

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.

Legal Information

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

Disclaimer

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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Storage Class

10 - Combustible liquids

flash_point_f

Not applicable

flash_point_c

Not applicable


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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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