Human alpha -Galactosidase A/GLA Antibody Summary
Accession # P06280
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.
alpha ‑Galactosidase A/GLA in A549 Human Cell Line. alpha ‑Galactosidase A/GLA was detected in immersion fixed A549 human lung carcinoma cell line using Mouse Anti-Human alpha ‑Galactosidase A/GLA Monoclonal Antibody (Catalog # MAB61461) at 8 µg/mL for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Mouse IgG Secondary Antibody (red; NL007) and counterstained with DAPI (blue). Specific staining was localized to cytoplasm. Staining was performed using our protocol for Fluorescent ICC Staining of Non-adherent Cells.
Preparation and Storage
- 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).
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.
Germain, D.P. (2002) Expert. Opin. Investig. Drugs. 11:1467.
Barngrover, D. (2003) J. Biotechnol. 95:280.
Mignani, R. and Cagnoli, L. (2004) J. Nephrol. 17:354.
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