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G4171

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

~1 mg/mL, affinity isolated antibody, buffered aqueous solution

Synonym(s):

Anti-D-glucosyl-N-acylsphingosine glucohydrolase, Anti-GBA, Anti-GBA1, Anti-Glucosidase, beta (Gluc), Anti-Glucosylceramidase (GlcCerase), Anti-Lysosomal glucocerebrosidase

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25 μL

$196.00

200 μL

$587.00

$196.00


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About This Item

NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
WB
Citations:
62

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biological source

rabbit

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

antigen ~60 kDa

species reactivity

human, mouse, rat

packaging

antibody small pack of 25 μL

enhanced validation

recombinant expression
Learn more about Antibody Enhanced Validation

concentration

~1 mg/mL

technique(s)

western blot: 1-2 μg/mL using HEK293-T cells lysate expressing human glucocerebrosidase (GBA)

UniProt accession no.

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Quality Level

Gene Information

human ... GBA(2629)
mouse ... Gba(14466)

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This Item
G4048M1946WH0002629M1
Quality Level

200

Quality Level

200

Quality Level

200

Quality Level

100

biological source

rabbit

biological source

rabbit

biological source

rabbit

biological source

mouse

conjugate

unconjugated

conjugate

unconjugated

conjugate

unconjugated

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody form

affinity isolated antibody

antibody form

affinity isolated antibody

antibody form

purified immunoglobulin

form

buffered aqueous solution

form

buffered aqueous solution

form

buffered aqueous solution

form

buffered aqueous solution

Gene Information

human ... GBA(2629)
mouse ... Gba(14466)

Gene Information

human ... SLC2A4(6517)
mouse ... Slc2a4(20528)
rat ... Slc2a4(25139)

Gene Information

human ... ASCC3(10973), MAOB(4129)
rat ... Maob(25750)

Gene Information

human ... GBA(2629)

General description

Glucocerebrosidase is an enzyme having glucosylceramidase activity. Defect in lysosomal hydrolase glucocerebrosidase results in Gaucher disease. Mutation in GBA will retard or block the transport of GBA to endoplasmic reticulum. Anti-glucocerebrosidase (C-terminal) antibody can be used to incubate the SDS-PAGE gel. Anti-Glucocerebrosidase antibody reacts specifically with human GBA.

Immunogen

synthetic peptide corresponding to amino acids 517-536 of human glucocerebrosidase (GBA), conjugated to KLH. This sequence is identical in rat GBA and highly conserved in mouse GBA (single amino acid substitution).

Application

Anti-Glucocerebrosidase (C-terminal) antibody produced in rabbit has been used in
  • immunohistochemistry
  • immunoblot analysis[1]

Anti-glucocerebrosidase (C-terminal) antibody can be used in western blotting and immunoblotting.

Biochem/physiol Actions

Glucocerebrosidase (GBA) activity is reduced in human with mutations in GBA gene and causes accumulation of glucosylceramide (GlcCer). Fibroblasts from patients with defined GBA mutations show either retarded or blocked transport of GBA in the endoplasmic reticulum. Mutations in the human GBA gene may contribute to the development of common age-related dementia known as dementia with Lewy bodies. Several studies indicate that mutations in the human GBA gene are associated with early-onset Parkinson disease.
Glucocerebrosidase is an enzyme having glucosylceramidase activity. Defect in lysosomal hydrolase glucocerebrosidase results in Gaucher disease. Mutation in GBA will retard or block the transport of GBA to endoplasmic reticulum.

Physical form

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

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

12 - Non Combustible Liquids

wgk

WGK 1

ppe

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


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Gaucher disease paradigm: From ERAD to comorbidity.
Inna Bendikov-Bar, Mia Horowitz
Human Mutation, 33(10), doi: 10-doi: 10 (2012)
Reducing GBA2 activity ameliorates neuropathology in Niemann-Pick type C mice
Marques ARA, et al.
Testing, 10(8), e0135889-e0135889 (2015)
N370S-GBA1 mutation causes lysosomal cholesterol accumulation in Parkinson's disease
Garcia-Sanz P, et al.
Movement Disorders, 32(10), 1409-1422 (2017)
Lysosomal enzyme glucocerebrosidase protects against Abeta1-42 oligomer-induced neurotoxicity
Choi S, et al.
Testing, 10(12), e0143854-e0143854 (2015)
Inna Bendikov-Bar et al.
Blood cells, molecules & diseases, 50(2), 141-145 (2012-11-20)
Gaucher disease (GD) is characterized by accumulation of glucosylceramide in lysosomes due to mutations in the GBA1 gene encoding the lysosomal hydrolase β-glucocerebrosidase (GCase). The disease has a broad spectrum of phenotypes, which were divided into three different Types; Type

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Instructions

Questions

  1. G4171 Anti-Glucocerebrosidase (C-terminal) antibody produced in rabbit Does this antibody cross react with GBA2 or GBA3?

    1 answer
    1. After performing a NCBI protein BLAST with the antibody immunogen sequence corresponding to amino acids 517-536 of human glucocerebrosidase (GBA) against either GBA2 or GBA3, there is 100% homology with GBA2 and 20% homology with GBA3. Therefore, binding with GBA2 is expected, while binding with GBA3 is not anticipated.

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