Overview

  • Product name

    Anti-GBA antibody [EPR5143(3)]
    See all GBA primary antibodies
  • Description

    Rabbit monoclonal [EPR5143(3)] to GBA
  • Host species

    Rabbit
  • Specificity

    The lab re-tested the antibody in mouse samples without obtaining satisfactory results, therefore we are not able to guarantee the antibody in this species. Please contact our Scientific Support if you have any feedback in mouse.
  • Tested applications

    Suitable for: WB, IHC-Pmore details
    Unsuitable for: Flow Cyt or IP
  • Species reactivity

    Reacts with: Rat, Human
  • Immunogen

    Synthetic peptide within Human GBA aa 500-600 (internal sequence). The exact sequence is proprietary.

  • Positive control

    • 293T, Saos-2, and U87-MG cell lysates, Human kidney tissue and Human thyroid carcinoma tissue
  • General notes

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated 'PUR' on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab128879 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/1000 - 1/10000. Predicted molecular weight: 60 kDa.

We suggest using 2% BSA/TBST as blocking buffer and antibody diluting buffer if you cannot obtain strong band in some samples.

IHC-P 1/100 - 1/200. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.

See IHC antigen retrieval protocols.

For unpurified use at 1/50.

  • Application notes
    Is unsuitable for Flow Cyt or IP.
  • Target

    • Involvement in disease

      Defects in GBA are the cause of Gaucher disease (GD) [MIM:230800]; also known as glucocerebrosidase deficiency. GD is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Different clinical forms are recognized depending on the presence (neuronopathic forms) or absence of central nervous system involvement, severity and age of onset.
      Defects in GBA are the cause of Gaucher disease type 1 (GD1) [MIM:230800]; also known as adult non-neuronopathic Gaucher disease. GD1 is characterized by hepatosplenomegaly with consequent anemia and thrombopenia, and bone involvement. The central nervous system is not involved.
      Defects in GBA are the cause of Gaucher disease type 2 (GD2) [MIM:230900]; also known as acute neuronopathic Gaucher disease. GD2 is the most severe form and is universally progressive and fatal. It manifests soon after birth, with death generally occurring before patients reach two years of age.
      Defects in GBA are the cause of Gaucher disease type 3 (GD3) [MIM:231000]; also known as subacute neuronopathic Gaucher disease. GD3 has central nervous manifestations.
      Defects in GBA are the cause of Gaucher disease type 3C (GD3C) [MIM:231005]; also known as pseudo-Gaucher disease or Gaucher-like disease.
      Defects in GBA are the cause of Gaucher disease perinatal lethal (GDPL) [MIM:608013]. It is a distinct form of Gaucher disease type 2, characterized by fetal onset. Hydrops fetalis, in utero fetal death and neonatal distress are prominent features. When hydrops is absent, neurologic involvement begins in the first week and leads to death within 3 months. Hepatosplenomegaly is a major sign, and is associated with ichthyosis, arthrogryposis, and facial dysmorphism.
      Note=Perinatal lethal Gaucher disease is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders.
      Defects in GBA contribute to susceptibility to Parkinson disease (PARK) [MIM:168600]. A complex neurodegenerative disorder characterized by bradykinesia, resting tremor, muscular rigidity and postural instability. Additional features are characteristic postural abnormalities, dysautonomia, dystonic cramps, and dementia. The pathology of Parkinson disease involves the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies (intraneuronal accumulations of aggregated proteins), in surviving neurons in various areas of the brain. The disease is progressive and usually manifests after the age of 50 years, although early-onset cases (before 50 years) are known. The majority of the cases are sporadic suggesting a multifactorial etiology based on environmental and genetic factors. However, some patients present with a positive family history for the disease. Familial forms of the disease usually begin at earlier ages and are associated with atypical clinical features.
    • Sequence similarities

      Belongs to the glycosyl hydrolase 30 family.
    • Cellular localization

      Lysosome membrane. Interaction with saposin-C promotes membrane association.
    • Information by UniProt
    • Database links

    • Alternative names

      • Acid beta glucosidase antibody
      • Acid beta-glucosidase antibody
      • Alglucerase antibody
      • Beta glucocerebrosidase antibody
      • BETA GLUCOSIDASE, ACID antibody
      • Beta-glucocerebrosidase antibody
      • betaGC antibody
      • D glucosyl N acylsphingosine glucohydrolase antibody
      • D-glucosyl-N-acylsphingosine glucohydrolase antibody
      • EC 3.2.1.45 antibody
      • GBA antibody
      • Gba protein antibody
      • GBA1 antibody
      • GC antibody
      • GCase antibody
      • GCB antibody
      • GLCM_HUMAN antibody
      • GLUC antibody
      • Glucocerebrosidase (alt.) antibody
      • Glucocerebrosidase antibody
      • GLUCOCEREBROSIDASE PSEUDOGENE antibody
      • Glucosidase beta antibody
      • Glucosidase, beta, acid antibody
      • Glucosidase, beta; acid (includes glucosylceramidase) antibody
      • Glucosylceramidase antibody
      • Imiglucerase antibody
      • Lysosomal glucocerebrosidase antibody
      • OTTHUMP00000033992 antibody
      • OTTHUMP00000033993 antibody
      see all

    Images

    • Lane 1: Wild-type HAP1 whole cell lysate (20 µg)
      Lane 2: GBA knockout HAP1 whole cell lysate (20 µg)
      Lane 3: MCF7 whole cell lysate (20 µg)
      Lane 4: HepG2 whole cell lysate (20 µg)
      Lanes 1 - 4: Merged signal (red and green). Green - ab128879 observed at 60 kDa. Red - loading control, ab8245, observed at 37 kDa.

      ab128879 was shown to specifically react with GBA in wild-type HAP1 cells as well as additional cross reactive bands. No bands were observed when GBA knockout samples were used. Wild-type and GBA knockout samples were subjected to SDS-PAGE. Ab128879 and ab8245 (Mouse anti GAPDH loading control) were incubated overnight at 4°C at 1/1000 dilution and 1/10,000 dilution respectively. Blots were developed with 800CW Goat anti-Rabbit and 680CW Goat anti-Mouse secondary antibodies at 1/10,000 dilution for 1 hour at room temperature before imaging.

    • Lane 1 : Anti-GBA antibody [EPR5143(3)] (ab128879) at 1/10000 dilution
      Lanes 2-4 : Anti-GBA antibody [EPR5143(3)] (ab128879) at 1/50000 dilution

      All lanes : C6 (Rat glial tumor glial cell) whole cell lysate

      Lysates/proteins at 20 µg per lane.

      Secondary
      All lanes : Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/100000 dilution

      Predicted band size: 60 kDa
      Observed band size: 60 kDa


      Exposure time: 180 seconds


      Blocking and diluting buffers:

      Lane 1 and 2: 5% NFDM/TBST
      Lane 3 and 4: 2% BSA/TBST

      We suggest using 2% BSA/TBST as blocking buffer and antibody diluting buffer if you cannot obtain strong band in some samples.

    • ab128879 staining GBA in Human kidney tissue sections by Immunohistochemistry (IHC-P - paraformaldehyde-fixed, paraffin-embedded sections). Tissue was fixed and paraffin-embedded, antigen retrieval was by heat mediation in Tris/EDTA buffer pH9. Samples were incubated with primary antibody (1/200). An undiluted HRP-conjugated anti-rabbit IgG was used as the secondary antibody. Tissue counterstained with Hematoxylin.

    • All lanes : Anti-GBA antibody [EPR5143(3)] (ab128879) at 1/4000 dilution

      Lane 1 : Saos-2 Cell Lysate
      Lane 2 : U87-MG Cell Lysate

      Lysates/proteins at 20 µg per lane.

      Secondary
      All lanes : Goat Anti-Rabbit IgG, (H+L), HRP- conjugated at 1/1000 dilution

      Predicted band size: 60 kDa

    • ab128879, unpurified, at a 1/100 dilution, staining GBA in paraffin embedded Human kidney tissue by Immunohistochemistry.

    • Equilibrium disassociation constant (KD)
      Learn more about KD

      Click here to learn more about KD
    • All lanes : Anti-GBA antibody [EPR5143(3)] (ab128879) at 1/1000 dilution (unpurified)

      Lane 1 : 293T cell lysate
      Lane 2 : Saos-2 cell lysate
      Lane 3 : U87-MG cell lysate

      Lysates/proteins at 10 µg per lane.

      Secondary
      All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution

      Predicted band size: 60 kDa

    • ab128879, unpurified, at a 1/100 dilution, staining GBA in paraffin embedded Human thyroid carcinoma tissue by Immunohistochemistry.

    References

    This product has been referenced in:

    • Malekkou A  et al. Biochemical Characterization of the GBA2 c.1780G>C Missense Mutation in Lymphoblastoid Cells from Patients with Spastic Ataxia. Int J Mol Sci 19:N/A (2018). Read more (PubMed: 30308956) »
    • Straniero L  et al. The GBAP1 pseudogene acts as a ceRNA for the glucocerebrosidase gene GBA by sponging miR-22-3p. Sci Rep 7:12702 (2017). Read more (PubMed: 28983119) »
    See all 4 Publications for this product

    Customer reviews and Q&As

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    1-2 of 2 Abreviews

    Application
    Western blot
    Sample
    Human Cell lysate - whole cell (HeLa cells)
    Gel Running Conditions
    Reduced Denaturing (4-12% gradient bis tris)
    Loading amount
    10 µg
    Treatment
    siRNA 40nM for 72 Hours
    Specification
    HeLa cells
    Blocking step
    Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 21°C

    Abcam user community

    Verified customer

    Submitted Nov 08 2018

    Application
    Immunocytochemistry/ Immunofluorescence
    Sample
    Human Cell (HeLa)
    Permeabilization
    Yes - Methanol fixation had no permeabilization prior to blocking. Paraformaldehyde fixation had either methanol permeabilization (-20oC, 10min) or no permeabilization prior to blocking. All samples were blocked with 0.1% saponin for 60min.
    Specification
    HeLa
    Blocking step
    BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 22°C
    Fixative
    4% Paraformaldehyde 10min or -20oC Methanol 10min

    Alastair Duly

    Verified customer

    Submitted Oct 09 2017

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