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Merck
CN

AV48882

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

NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
IHC, WB
Citations:
1

Key Documents

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biological source

rabbit

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

Quality Level

100

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.

Physical form

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

Other Notes

Synthetic peptide located within the following region: RCRVGQWVRMENVTVLGEDVIVNDELYLNGASVLPHKSIGESVPEPRIIM

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

10 - Combustible liquids

wgk

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

Regulatory Information

低风险生物材料
常规特殊物品
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Certificates of Analysis (COA)

Lot/Batch Number
QC18196

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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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