Recombinant
RabMAb

Anti-Galactosidase alpha antibody [EPR5829] (ab129173)

Overview

  • Product name
    Anti-Galactosidase alpha antibody [EPR5829]
    See all Galactosidase alpha primary antibodies
  • Description
    Rabbit monoclonal [EPR5829] to Galactosidase alpha
  • Host species
    Rabbit
  • Tested applications
    Suitable for: WBmore details
    Unsuitable for: Flow Cyt,ICC,IHC-P or IP
  • Species reactivity
    Reacts with: Human
    Does not react with: Mouse, Rat
  • Immunogen

    Synthetic peptide within Human Galactosidase alpha aa 400-500 (C terminal). The exact sequence is proprietary.

  • Positive control
    • WB: HEK-293T, A431 and MCF7 cell lysates.
  • 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

  • Form
    Liquid
  • Storage instructions
    Shipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
  • Dissociation constant (KD)
    KD = 4.49 x 10 -11 M
    Learn more about KD
  • Storage buffer
    pH: 7.20
    Preservative: 0.01% Sodium azide
    Constituents: 49% PBS, 50% Glycerol, 0.05% BSA
  • Concentration information loading...
  • Purity
    Protein A purified
  • Clonality
    Monoclonal
  • Clone number
    EPR5829
  • Isotype
    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab129173 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. Detects a band of approximately 46 kDa (predicted molecular weight: 49 kDa).
  • Application notes
    Is unsuitable for Flow Cyt,ICC,IHC-P or IP.
  • Target

    • Involvement in disease
      Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.
    • Sequence similarities
      Belongs to the glycosyl hydrolase 27 family.
    • Cellular localization
      Lysosome.
    • Information by UniProt
    • Database links
    • Alternative names
      • AGAL_HUMAN antibody
      • Agalsidase alfa antibody
      • Alpha D galactosidase A antibody
      • Alpha D galactoside galactohydrolase 1 antibody
      • Alpha D galactoside galactohydrolase antibody
      • Alpha gal A antibody
      • Alpha galactosidase A antibody
      • Alpha-D-galactosidase A antibody
      • Alpha-D-galactoside galactohydrolase antibody
      • Alpha-galactosidase A antibody
      • GALA antibody
      • Galactosidase, alpha antibody
      • GLA antibody
      • GLA protein antibody
      • Melibiase antibody
      see all

    Images

    • All lanes : Anti-Galactosidase alpha antibody [EPR5829] (ab129173) at 1/1000 dilution

      Lane 1 : 293T cell lysate
      Lane 2 : A431 cell lysate
      Lane 3 : MCF7 cell lysate

      Lysates/proteins at 10 µg per lane.

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

      Predicted band size: 49 kDa

    • Equilibrium disassociation constant (KD)
      Learn more about KD

      Click here to learn more about KD

    References

    This product has been referenced in:
    • Vieitez I  et al. Fabry disease in the Spanish population: observational study with detection of 77 patients. Orphanet J Rare Dis 13:52 (2018). WB ; Human . Read more (PubMed: 29631605) »

    See 1 Publication for this product

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    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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