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
Sterile PBS to a final concentration of 0.2 mg/mL.
Reconstitution Buffer Available
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 -GalactosidaseA 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 storagedisease with the accumulation of Gb3 in the walls of small blood vessels,nerves, dorsal root ganglia, renal glomerular and tubular epithelialcells, 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).
Ioannou, Y.A. et al. (1998) Biochem. J. 332:789.
Koide, T. et al. (1990) FEBS Lett. 259:353.
Ioannou Y.A, et al. (1992) J. Cell Biol. 119:1137.
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.