Overview

  • Product name

    Anti-GBA antibody [EPR5142] - BSA and Azide free
    See all GBA primary antibodies
  • Description

    Rabbit monoclonal [EPR5142] to GBA - BSA and Azide free
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, WBmore details
    Unsuitable for: Flow Cyt,ICC or IP
  • Species reactivity

    Reacts with: Human
    Does not react with: Mouse, Rat
  • Immunogen

    Synthetic peptide within Human GBA aa 50-150. The exact sequence is proprietary.

  • Positive control

    • IHC-P: Human lung cancer and colon tissue; WB: HAP1, MCF7 and HepG2 cell lysates.
  • General notes

    Ab215261 is the carrier-free version of ab125065. This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.

     

    Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.

    Use our conjugation kits  for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.

    ab215261 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.

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

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab215261 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
IHC-P Use at an assay dependent concentration. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
WB Use at an assay dependent concentration. Detects a band of approximately 60 kDa (predicted molecular weight: 60 kDa).
  • Application notes
    Is unsuitable for Flow Cyt,ICC 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 (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 - ab125065 observed at 70 kDa. Red - loading control, ab8245, observed at 37 kDa.

      ab125065 was shown to specifically recognize 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. Ab125065 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 Goat anti-Rabbit IgG H&L (IRDye® 800CW) preabsorbed (ab216773) and Goat anti-Mouse IgG H&L (IRDye® 680RD) preabsorbed (ab216776) secondary antibodies at 1/10,000 dilution for 1 hour at room temperature before imaging.

      This data was developed using the same antibody clone in a different buffer formulation containing tissue culture supernatant, tris glycine, BSA, glycerol and sodium azide (ab125065).

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of Human lung cancer tissue sections labeling GBA with Purified ab125065 at 1:50 dilution (2.1 µg/ml). Heat mediated antigen retrieval was performed Perform heat mediated antigen retrieval using ab93684 (Tris/EDTA buffer, pH 9.0). ImmunoHistoProbe one step HRP Polymer (ready to use)was used as the secondary antibody. Negative control:PBS instead of the primary antibody. Hematoxylin was used as a counterstain.

      This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab125065). 

       

    • ab125065, at a 1/50 dilution, staining GBA in paraffin-embedded Human colon tissue by immunohistochemistry.

      This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab125065). 

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

    References

    ab215261 has not yet been referenced specifically in any publications.

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