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Glucosylceramidase/GBA Antibody (2E2)

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Western Blot: Glucosylceramidase/GBA Antibody (2E2) [H00002629-M01] - Effect of Diltiazem on L444P and N370S/V394L GC Folding and Trafficking.Western blot analysis of untreated and diltiazem-treated L444P GC cells. ...read more
Immunocytochemistry/ Immunofluorescence: Glucosylceramidase/GBA Antibody (2E2) [H00002629-M01] - Analysis of monoclonal antibody to GBA on HeLa cell. Antibody concentration 10 ug/ml.
Immunohistochemistry-Paraffin: Glucosylceramidase/GBA Antibody (2E2) [H00002629-M01] - Analysis of monoclonal antibody to GBA on formalin-fixed paraffin-embedded human breast cancer. Antibody concentration 3 ug/ml.
Western Blot: Glucosylceramidase/GBA Antibody (2E2) [H00002629-M01] - GBA monoclonal antibody (M01), clone 2E2 Analysis of GBA expression in MCF-7.
Western Blot: Glucosylceramidase/GBA Antibody (2E2) [H00002629-M01] - Analysis of GBA expression in transfected 293T cell line by GBA monoclonal antibody (M01), clone 2E2.Lane 1: GBA transfected lysate(60 KDa).Lane 2: ...read more
ELISA: Glucosylceramidase/GBA Antibody (2E2) [H00002629-M01] - Glucosylceramidase/GBA Antibody (2E2) [H00002629-M01] - Detection limit for recombinant GST tagged GBA is approximately 1ng/ml as a capture antibody.
Effect of Diltiazem on L444P and N370S/V394L GC Folding and Trafficking(A) Western blot analysis of untreated and diltiazem-treated L444P GC cells. L444P GC cells were cultured without or with diltiazem at varying ...read more

Product Details

Summary
Reactivity Hu, PlSpecies Glossary
Applications WB, ELISA, ICC/IF, IHC, IP
Clone
2E2
Clonality
Monoclonal
Host
Mouse
Conjugate
Unconjugated

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Glucosylceramidase/GBA Antibody (2E2) Summary

Description
Quality control test: Antibody Reactive Against Recombinant Protein.
Immunogen
GBA (NP_000148, 146 a.a. ~ 235 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. SYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGS
Specificity
GBA - glucosidase, beta; acid (includes glucosylceramidase)
Isotype
IgG2a Kappa
Clonality
Monoclonal
Host
Mouse
Gene
GBA
Purity
IgG purified
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.

Applications/Dilutions

Dilutions
  • ELISA
  • Immunocytochemistry/ Immunofluorescence 10ug/ml
  • Immunohistochemistry 3ug/ml
  • Immunohistochemistry-Paraffin 3ug/ml
  • Immunoprecipitation
  • Western Blot 1:500
Application Notes
Antibody reactivity against recominant protein, transfected lysate and cell lysate for WB. It has been used for IF, IHC-P and ELISA.
Reviewed Applications
Read 1 Review rated 5
using
H00002629-M01 in the following applications:

Publications
Read Publications using
H00002629-M01 in the following applications:

  • WB
    2 publications

Reactivity Notes

Plant reactivity reported in scientific literature (PMID: 22592100).

Packaging, Storage & Formulations

Storage
Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
Buffer
In 1x PBS, pH 7.4
Preservative
No Preservative
Purity
IgG purified

Notes

This product is produced by and distributed for Abnova, a company based in Taiwan.

Alternate Names for Glucosylceramidase/GBA Antibody (2E2)

  • Acid beta-glucosidase
  • Alglucerase
  • beta-glucocerebrosidase
  • D-glucosyl-N-acylsphingosine glucohydrolase
  • EC 3.2.1.45
  • GBA
  • GBA1
  • GC
  • GCB
  • GLUC
  • glucosidase, beta, acid
  • glucosidase, beta; acid (includes glucosylceramidase)
  • Glucosylceramidase
  • Imiglucerase
  • lysosomal glucocerebrosidase

Background

This gene encodes a lysosomal membrane protein that cleaves the beta-glucosidic linkage of glycosylceramide, an intermediate in glycolipid metabolism. Mutations in this gene cause Gaucher disease, a lysosomal storage disease characterized by an accumulation of glucocerebrosides. A related pseudogene is approximately 12 kb downstream of this gene on chromosome 1. Alternative splicing results in multiple transcript variants encoding the same protein.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Applications: WB, ELISA, ICC/IF, IHC, IP

Publications for Glucosylceramidase/GBA Antibody (H00002629-M01)(10)

We have publications tested in 2 confirmed species: Human, Plant.

We have publications tested in 1 application: WB.


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WB
(2)
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Human
(1)
Plant
(1)
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Showing Publications 1 - 10 of 10.
Publications using H00002629-M01 Applications Species
Jan Philipp Dobert, Simon Bub, Rebecca Mächtel, Dovile Januliene, Lisa Steger, Martin Regensburger, Sibylle Wilfling, Jia-Xuan Chen, Mario Dejung, Sonja Plötz, Ute Hehr, Arne Moeller, Philipp Arnold, Friederike Zunke Activation and Purification of beta -Glucocerebrosidase by Exploiting its Transporter LIMP-2 - Implications for Novel Treatment Strategies in Gaucher's and Parkinson's Disease. Advanced science (Weinheim, Baden-Wurttemberg, Germany) 2024-04-26 [PMID: 38666485]
Jung J, Choi H, Huy N et al. Production of recombinant human acid ?-glucosidase with high mannose-type N-glycans in rice gnt1 mutant for potential treatment of Gaucher disease. Protein Expr Purif. 2019-02-27 [PMID: 30822514]
Atashrazm F, Hammond D, Perera G et al. Reduced glucocerebrosidase activity in monocytes from patients with Parkinson's disease. Sci Rep. 2018-10-18 [PMID: 30337601]
Bendikov-Bar I, Rapaport D, Larisch S, Horowitz M. Parkin-mediated ubiquitination of mutant glucocerebrosidase leads to competition with its substrates PARIS and ARTS. Orphanet J Rare Dis. 2014-06-16 [PMID: 24935484]
Murphy Karen E, Gysbers Amanda M, Abbott Sarah K et al. Reduced glucocerebrosidase is associated with increased alpha-synuclein in sporadic Parkinson's disease. Brain. 2014-01-28 [PMID: 24477431] (WB, Human) WB Human
Bendikov-Bar I, Horowitz M. Gaucher disease paradigm: From ERAD to comorbidity. Hum Mutat. 2012-06-11 [PMID: 22623374]
Gholamreza Babajani, Michael B Tropak, Don Mahuran, Allison R Kermode. Pharmacological chaperones facilitate the post-ER transport of recombinant N370S mutant ?-glucocerebrosidase in plant cells: Evidence that N370S is a folding mutant, . Molecular Genetics and Metabolism, 10.1016/j.ymgme.04.018. 2012-01-01 [PMID: 22592100] (WB, Plant) WB Plant
Bendikov-Bar I, Ron I, Filocamo M, Horowitz M. Characterization of the ERAD process of the L444P mutant glucocerebrosidase variant. Blood Cells Mol Dis 46(1):4-10. 2011-01-15 [PMID: 21106416]
Campeau PM, Rafei M, Boivin MN et al. Characterization of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome. Blood. 2009-10-08 [PMID: 19587377]
Ting-Wei Mu, Douglas M Fowler, Jeffery W Kelly, Peter Walter Partial Restoration of Mutant Enzyme Homeostasis in Three Distinct Lysosomal Storage Disease Cell Lines by Altering Calcium Homeostasis PLoS Biology 2008-02-01 [PMID: 18254660]

Review for Glucosylceramidase/GBA Antibody (H00002629-M01) (1) 51

Average Rating: 5
(Based on 1 review)
We have 1 review tested in 1 species: ansgenic mice.

Reviews using H00002629-M01:
Filter by Applications
WB
(1)
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Filter by Species
ansgenic mice
(1)
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Images Ratings Applications Species Date Details
Western Blot Glucosylceramidase/GBA H00002629-M01
Enlarge
5
reviewed by:
Steven Zhou
WB ansgenic mice 10/10/2016
View

Summary

ApplicationWestern Blot
Sample TestedMouse brain,mouse liver,Mouse Spleen
Speciesansgenic mice
LotG9051-2E2

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ICC/IF Video Protocol

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Recent Reviews

5
5
1
4
0
3
0
2
0
1
0

Steven Zhou
10/10/2016
Application: WB
Species: ansgenic mice

Bioinformatics

Gene Symbol GBA
Entrez
OMIM
Uniprot