Human alpha -Galactosidase A/GLA Antibody

  
  • 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 as a 0.2 µm filtered solution in PBS.
  • Label
    Unconjugated
Applications
  •  
    Recommended
    Concentration
    Sample
  • 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.
Data Examples
Immunocytochemistry
alpha ‑Galactosidase A/GLA in HeLa Human Cell Line. alpha ‑Galactosidase A/GLA was detected in immersion fixed HeLa human cervical epithelial carcinoma cell line using Sheep Anti-Human alpha ‑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 Northern­Lights™ 557-conjugated Anti-Sheep IgG Secondary Antibody (red; Catalog # NL010). LAMP1 was also detected using Mouse Anti-Human LAMP1 Mono­clonal Antibody (Catalog # MAB4800). Cells were stained using the Northern­Lights™ 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.
Preparation and Storage
  • Reconstitution
    Sterile PBS to a final concentration of 0.2 mg/mL.
  • 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
Related Research Areas
Cell and Tissue Staining Kits
Description Application Cat# Citations Images  

Anti-Sheep HRP-DAB Cell & Tissue Staining Kit

CTS019 1
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....
Isotype Controls
Description Application Cat# Citations Images  

Normal Sheep IgG Control

Ctrl 5-001-A 9
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....
Secondary Antibodies
Description Application Cat# Citations Images  

Sheep IgG HRP-conjugated Antibody

WB, Simple Western HAF016 14  
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG NorthernLights™ NL557-conjugated Antibody

Flow, IHC, ICC NL010 3
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG NorthernLights™ NL637-conjugated Antibody

Flow, IHC, ICC NL011 5
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG NorthernLights™ NL493-conjugated Antibody

Flow, IHC, ICC NL012 2
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Sheep IgG (H+L) PE-conjugated Antibody

Flow F0126  
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG Biotinylated Antibody

WB BAF016 2
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Sheep IgG (H+L) APC-conjugated Antibody

Flow F0127  
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Sheep IgG VisUCyte HRP Polymer Antibody

IHC VC006  
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG (H+L) Fluorescein-conjugated Antibody

Flow F0125 1
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG (H+L) PerCP-conjugated Antibody

Flow F0128
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Have you used Human alpha -Galactosidase A/GLA Antibody?

Submit a review and receive a $25US/€18/£15/$25CAN amazon gift card if you include an image - $10US/€7/£6/$10CAN Amazon card for reviews without an image. Limited to verified customers in USA, Canada and Europe.

Order Details

Contact Information

Calculators

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.

=
÷
R&D Systems Guarantee

Monoclonal
Antibody
Panels For
Screening

Learn More
Monoclonal Antibodies

Feedback

Customer Information