Human alpha -Galactosidase A/GLA Antibody

Recombinant Monoclonal Antibody
Catalog # Availability Size / Price Qty
Detection of Human  alpha ‑Galactosidase A/GLA by Western Blot.
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Product Details

Human alpha -Galactosidase A/GLA Antibody Summary

Species Reactivity
Detects human alpha-Galactosidase A/GLA in direct ELISA.
Recombinant Monoclonal Rabbit IgG Clone # 2520B
Protein A or G purified from hybridoma culture supernatant
Human alpha-Galactosidase A/GLA
Accession # P06280
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.

Scientific Data

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, MCF‑7 human breast cancer cell line and NCI‑H460 human large cell lung carcinoma cell line. PVDF membrane was probed with 1 µg/mL of Rabbit Anti-Human alpha ‑Galactosidase A/GLA Monoclonal Antibody (Catalog # MAB61462) followed by HRP-conjugated Anti-Rabbit IgG Secondary Antibody (Catalog # HAF008). A specific band was detected for alpha ‑Galactosidase A/GLA at approximately 45 kDa (as indicated). This experiment was conducted under reducing conditions and using Western Blot Buffer Group 1.

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Preparation and Storage

Reconstitute at 0.5 mg/mL in sterile PBS.
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).

  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; GALA; galactosidase, alpha; GLA; Melibiase

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