Overview

  • Product name

    Anti-Galactosidase alpha antibody
    See all Galactosidase alpha primary antibodies
  • Description

    Rabbit polyclonal to Galactosidase alpha
  • Host species

    Rabbit
  • Tested applications

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

    Reacts with: Human
  • Immunogen

    Recombinant fragment corresponding to Human Galactosidase alpha aa 150-429. (NP_000160.1).
    Sequence:

    GYYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIV YSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFN QERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSND LRHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAW AVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEW TSRLRSHINPTGTVLLQLENTMQMSLKDLL


    Database link: P06280

  • Positive control

    • WB: SW620, BT-474 and HeLa cell extracts. IHC-P: Human adrenal tissue. ICC/IF: HeLa cells.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    pH: 7.30
    Preservative: 0.02% Sodium azide
    Constituents: PBS, 50% Glycerol
  • Concentration information loading...
  • Purity

    Affinity purified
  • Clonality

    Polyclonal
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab230924 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 1/500 - 1/2000. Predicted molecular weight: 49 kDa.
IHC-P 1/100 - 1/200. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
ICC/IF 1/50 - 1/100.

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 (ab230924) at 1/500 dilution

    Lane 1 : SW620 cell extract
    Lane 2 : BT-474 (human ductal breast epithelial tumor cell line) cell extract
    Lane 3 : HeLa (human epithelial cell line from cervix adenocarcinoma) cell extract

    Predicted band size: 49 kDa

  • Formalin-fixed, paraffin-embedded human adrenal tissue stained for Galactosidase alpha with ab230924 at 1/100 dilution in immunohistochemical analysis.

  • HeLa (human epithelial cell line from cervix adenocarcinoma) cells stained for Galactosidase alpha (green) using ab230924 at 1/50 dilution in ICC/IF. 

    The nuclear counter stain is DAPI (blue).

References

ab230924 has not yet been referenced specifically in any publications.

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