Anti-GBA antibody (ab55080)

Knockout Tested Mouse monoclonal GBA antibody. Validated in WB, IHC, ICC/IF and tested in Human. Cited in 7 publication(s). Independently reviewed in 3 review(s).

Overview

  • Product name

  • Description

    Mouse monoclonal to GBA
  • Host species

    Mouse
  • Tested applications

    Suitable for: WB, IHC-P, IHC-Fr, ICC/IFmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Recombinant fragment (GST-tag) corresponding to Human GBA aa 146-236.
    Sequence:

    SYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKL KIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGS

  • General notes

    This product was changed from ascites to tissue culture supernatant on 15 May 2019. Please note that the dilutions may need to be adjusted accordingly. If you have any questions, please do not hesitate to contact our scientific support team.

Properties

Applications

Our Abpromise guarantee covers the use of ab55080 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 Use at an assay dependent concentration. Predicted molecular weight: 60 kDa.
IHC-P Use at an assay dependent concentration.
IHC-Fr Use at an assay dependent concentration.
ICC/IF Use at an assay dependent concentration.

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 (40 µg)
    Lane 2: GBA knockout HAP1 whole cell lysate (40 µg)
    Lane 3: MCF7 whole cell lysate (40 µg)
    Lane 4: HepG2 whole cell lysate (40 µg) 

    Lanes 1 - 4: Merged signal (red and green). Green - ab55080 observed at 70 kDa. Red - loading control, ab181602, observed at 37 kDa. 

    ab55080 was shown to specifically react with GBA in wild-type HAP1 cells along with 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. Ab55080 and ab181602 (Rabbit anti GAPDH loading control) were incubated overnight at 4°C at 1 ug/ml and 1/10,000 dilution respectively. Blots were developed with Goat anti-Mouse IgG H&L (IRDye® 800CW) preabsorbed (ab216772) and Goat anti-Rabbit IgG H&L (IRDye® 680RD) preabsorbed (ab216777) secondary antibodies at 1/10,000 dilution for 1 hour at room temperature before imaging.

    This image was generated using the ascites version of the product.

  • ab55080 at 10 ug/ml staining GBA in human Hela cells by Immunocytochemistry/ Immunofluorescence.

    This image was generated using the ascites version of the product.

  • GBA antibody (ab55080) at 1ug/lane + MCF-7 cell lysate at 25ug/lane.

    This image was generated using the ascites version of the product.

  • GBA antibody (ab55080) used in immunohistochemistry at 3ug/ml on formalin fixed and paraffin embedded human breast cancer.

    This image was generated using the ascites version of the product.

  • All lanes : Anti-GBA antibody (ab55080) at 1/1000 dilution

    Lane 1 : Lysate prepared from MOCK
    Lane 2 : Lysate prepared from human HN10 cells

    Lysates/proteins at 10 µg per lane.

    Secondary
    All lanes : IRDye® donkey polyclonal to mouse IgG at 1/3000 dilution

    Performed under reducing conditions.

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


    Exposure time: 1 minute


    This image was generated using the ascites version of the product.

    See Abreview

References

This product has been referenced in:

  • Drews K  et al. Glucosylceramidase Maintains Influenza Virus Infection by Regulating Endocytosis. J Virol 93:N/A (2019). Read more (PubMed: 30918081) »
  • Dopeso-Reyes IG  et al. Glucocerebrosidase expression patterns in the non-human primate brain. Brain Struct Funct 223:343-355 (2018). IHC-FrFl . Read more (PubMed: 28835999) »
See all 8 Publications for this product

Customer reviews and Q&As

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1-3 of 3 Abreviews

Application
Western blot
Sample
Rat Tissue lysate - whole (Brain)
Loading amount
20 µg
Specification
Brain
Gel Running Conditions
Reduced Denaturing (10% SDS-PAGE)
Blocking step
Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 24°C

Dr. Ruma Raha-Chowdhury

Verified customer

Submitted Sep 07 2010

Application
Immunohistochemistry (Frozen sections)
Sample
Human Tissue sections (Brain section)
Specification
Brain section
Fixative
Paraformaldehyde
Permeabilization
Yes - 0.1x PBS plus 0.3xTriton x
Blocking step
Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 24°C

Dr. Ruma Raha-Chowdhury

Verified customer

Submitted Sep 06 2010

Application
Western blot
Sample
Human Cell lysate - whole cell (HN10 cells)
Loading amount
10 µg
Specification
HN10 cells
Treatment
transfected with pcDNA-GBA
Gel Running Conditions
Reduced Denaturing (4-20%)
Blocking step
BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C

Abcam user community

Verified customer

Submitted Jun 10 2009

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