Human alpha -Galactosidase A/GLA Antibody

    
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  • 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
Product Datasheets

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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 Example
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.
    Reconstitution Buffer Available
  • 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

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