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Key Documents

Safety Information

AV48882

Sigma-Aldrich

Anti-GMPPB antibody produced in rabbit

IgG fraction of antiserum

Synonym(s):

Anti-GDP-mannose pyrophosphorylase B, Anti-KIAA1851

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

biological source

rabbit

Quality Level

100

conjugate

unconjugated

antibody form

IgG fraction of antiserum

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

43 kDa

species reactivity

human, rabbit, guinea pig, mouse, horse, dog, bovine, rat

concentration

0.5 mg - 1 mg/mL

technique(s)

immunohistochemistry: suitable
western blot: suitable

NCBI accession no.

NP_068806

UniProt accession no.

Q9Y5P6-2

shipped in

wet ice

storage temp.

−20°C

Gene Information

human ... GMPPB(29925)

Immunogen

Synthetic peptide directed towards the C terminal region of human GMPPB

Application

Anti-GMPPB antibody produced in rabbit is suitable for western blotting at a concentration of 5.0μg/ml and for immunohistochemistry of paraffin-embedded tissue sections at a concentration of 4-8μg/ml.

Biochem/physiol Actions

GDP-mannose pyrophosphorylase B (GMPPB) catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose during the synthesis of N-linked oligosaccharides. Mutations in GMPPB gene results in hypoglycosylation of α-dystroglycan characteristic of congenital and limb-girdle muscular dystrophies.

Sequence

Synthetic peptide located within the following region: RCRVGQWVRMENVTVLGEDVIVNDELYLNGASVLPHKSIGESVPEPRIIM

Physical form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), 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 Code

10 - Combustible liquids

WGK

WGK 3

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Regulatory Listings

Regulatory Listings are mainly provided for chemical products. Only limited information can be provided here for non-chemical products. No entry means none of the components are listed. It is the user’s obligation to ensure the safe and legal use of the product.

JAN Code

AV48882-100UL:
AV48882-100UG:

Certificates of Analysis (COA)

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Keren J Carss et al.
American journal of human genetics, 93(1), 29-41 (2013-06-19)
Congenital muscular dystrophies with hypoglycosylation of α-dystroglycan (α-DG) are a heterogeneous group of disorders often associated with brain and eye defects in addition to muscular dystrophy. Causative variants in 14 genes thought to be involved in the glycosylation of α-DG

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