Human alpha -Galactosidase A/GLA Antibody

Catalog # Availability Size / Price Qty
AF6146
AF6146-SP
Detection of Human  alpha ‑Galactosidase A/GLA by Western Blot.
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Product Details
Citations (1)
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Human alpha -Galactosidase A/GLA Antibody Summary

Species Reactivity
Human
Specificity
Detects human alpha ‑Galactosidase A/GLA in direct ELISAs.
Source
Polyclonal Sheep IgG
Purification
Antigen Affinity-purified
Immunogen
Chinese hamster ovary cell line CHO-derived recombinant human alpha ‑Galactosidase A/GLA
Leu32-Leu429
Accession # P06280
Formulation
Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.
Label
Unconjugated

Applications

Recommended Concentration
Sample
Western Blot
2 µg/mL
See below
Simple Western
50 µg/mL
See below
Immunocytochemistry
5-15 µg/mL
See below

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Scientific Data

Western Blot Detection of Human a-Galactosidase A/GLA antibody by Western Blot. View Larger

Detection of Human alpha ‑Galactosidase A/GLA by Western Blot. Western blot shows lysates of A549 human lung carcinoma cell line, HeLa human cervical epithelial carcinoma cell line, and HepG2 human hepatocellular carcinoma cell line. PVDF membrane was probed with 2 µg/mL of Sheep Anti-Human a-Galactosidase A/GLA Antigen Affinity-purified Polyclonal Antibody (Catalog # AF6146) followed by HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # HAF016). A specific band was detected for a-Galactosidase A/GLA at approximately 38 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

Immunocytochemistry a-Galactosidase A/GLA antibody in HeLa Human Cell Line by Immunocytochemistry (ICC). View Larger

alpha ‑Galactosidase A/GLA in HeLa Human Cell Line. a-Galactosidase A/GLA was detected in immersion fixed HeLa human cervical epithelial carcinoma cell line using Sheep Anti-Human a-Galactosidase A/GLA Antigen Affinity-purified Polyclonal Antibody (Catalog # AF6146) at 15 µg/mL for 3 hours at room temperature. Cells were stained using the Northern-Lights™ 557-conjugated Anti-Sheep IgG Secondary Antibody (red; Catalog # NL010). LAMP1 was also detected using Mouse Anti-Human LAMP1 Mono-clonal Antibody (Catalog # MAB4800). Cells were stained using the Northern-Lights™ 493-conjugated Anti-Mouse IgG Secondary Antibody (green; Catalog # NL009). Cells were counterstained with DAPI (blue). Specific staining was localized to lysosomes. View our protocol for Fluorescent ICC Staining of Cells on Coverslips.

Simple Western Detection of Human a-Galactosidase A/GLA antibody by Simple Western<sup>TM</sup>. View Larger

Detection of Human alpha ‑Galactosidase A/GLA by Simple WesternTM. Simple Western lane view shows lysates of MCF-7 human breast cancer cell line and HeLa human cervical epithelial carcinoma cell line, loaded at 0.2 mg/mL. A specific band was detected for a-Galactosidase A/GLA at approximately 55 kDa (as indicated) using 50 µg/mL of Sheep Anti-Human a-Galactosidase A/GLA Antigen Affinity-purified Polyclonal Antibody (Catalog # AF6146) followed by 1:50 dilution of HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # HAF016). This experiment was conducted under reducing conditions and using the 12-230 kDa separation system.

Reconstitution Calculator

Reconstitution Calculator

The reconstitution calculator allows you to quickly calculate the volume of a reagent to reconstitute your vial. Simply enter the mass of reagent and the target concentration and the calculator will determine the rest.

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Preparation and Storage

Reconstitution
Sterile PBS to a final concentration of 0.2 mg/mL.
Reconstitution Buffer Available
Reconstitution Buffer 1 (PBS)
Catalog #
Availability
Size / Price
Qty
RB01
Shipping
The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
Stability & Storage
Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
  • 6 months, -20 to -70 °C under sterile conditions after reconstitution.

Background: alpha-Galactosidase A/GLA

Human alpha -Galactosidase A is a homodimeric glycoprotein that can release terminal alpha -galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose (1). It is a lysosomal enzyme and is responsible for degradation of glycolipid globotriaosylceramide (Gb3) (Gal alpha 1‑4Gal beta 1‑4Glc beta ‑ceramide). Mutations in this gene cause Fabry disease, an X-linked hereditary lysosomal storage disease with the accumulation of Gb3 in the walls of small blood vessels, nerves, dorsal root ganglia, renal glomerular and tubular epithelial cells, and cardiomyocytes (2, 3). Inability to prevent the glycosphingolipid deposition can cause hypertension, strokes, heart attack and progressive renal failure (4). Current treatment for Fabry disease is enzyme replacement therapy using intravenously delivered recombinant alpha -Galactosidase A (5, 6).

References
  1. Ioannou, Y.A. et al. (1998) Biochem. J. 332:789.
  2. Koide, T. et al. (1990) FEBS Lett. 259:353.
  3. Ioannou Y.A, et al. (1992) J. Cell Biol. 119:1137.
  4. Germain, D.P. (2002) Expert. Opin. Investig. Drugs. 11:1467.
  5. Barngrover, D. (2003) J. Biotechnol. 95:280.
  6. Mignani, R. and Cagnoli, L. (2004) J. Nephrol. 17:354.
Entrez Gene IDs
2717 (Human)
Alternate Names
agalsidase alfa; Agalsidase alpha; Agalsidase; Alpha-D-galactosidase A; alpha-D-galactoside galactohydrolase 1; Alpha-D-galactoside galactohydrolase; alpha-gal A; alpha-galactosidase A; EC 3.2.1; EC 3.2.1.22; GALA; galactosidase, alpha; GLA; Melibiase

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Citation for Human alpha -Galactosidase A/GLA Antibody

R&D Systems personnel manually curate a database that contains references using R&D Systems products. The data collected includes not only links to publications in PubMed, but also provides information about sample types, species, and experimental conditions.

1 Citation: Showing 1 - 1

  1. AAV2/6 Gene Therapy in a Murine Model of Fabry Disease Results in Supraphysiological Enzyme Activity and Effective Substrate Reduction
    Authors: M Yasuda, MW Huston, S Pagant, L Gan, S St Martin, S Sproul, D Richards, S Ballaron, K Hettini, A Ledeboer, L Falese, L Cao, Y Lu, MC Holmes, K Meyer, RJ Desnick, T Wechsler
    Mol Ther Methods Clin Dev, 2020;18(0):607-619.
    Species: Mouse
    Sample Types: Protein
    Applications: Western Blot

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