Overview

  • Product name
    Anti-Galactosidase alpha antibody
    See all Galactosidase alpha primary antibodies
  • Description
    Rabbit polyclonal to Galactosidase alpha
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Non human primates
  • Immunogen

    Synthetic peptide conjugated to KLH derived from within residues 1 - 100 of Human Galactosidase alpha.

    (Peptide available as ab91202.)

  • Positive control
    • This antibody gave a positive signal in Human Liver Tissue Lysate.

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 or -80°C. Avoid freeze / thaw cycle.
  • Storage buffer
    Preservative: 0.02% Sodium Azide
    Constituents: 1% BSA, PBS, pH 7.4
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab70520 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 a concentration of 1 µg/ml. Detects a band of approximately 49 kDa (predicted molecular weight: 49 kDa).

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

  • Anti-Galactosidase alpha antibody (ab70520) at 1 µg/ml + Human liver tissue lysate - total protein (ab29889) at 10 µg

    Secondary
    Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 49 kDa
    Observed band size : 49 kDa
    Additional bands at : 47 kDa (possible cleavage fragment).

    Galactosidase alpha contains a 31 amino acid signal peptide. We hypothesize that the 47 kDa band represents the mature protein. Some batches of this product also recognise a band at 100 kDa in Western Blot, we are unsure of the identity of this band.

References

This product has been referenced in:
  • Citro V  et al. Identification of an Allosteric Binding Site on Human Lysosomal Alpha-Galactosidase Opens the Way to New Pharmacological Chaperones for Fabry Disease. PLoS One 11:e0165463 (2016). WB . Read more (PubMed: 27788225) »
  • Sienkiewicz AE  et al. Aberrant glycosylation in the human trabecular meshwork. Proteomics Clin Appl 8:130-42 (2014). ELISA . Read more (PubMed: 24458570) »

See all 3 Publications 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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