Human alpha -Galactosidase A/GLA Antibody

Catalog #: MAB61462 Datasheet / COA / SDS

Recombinant Monoclonal Antibody

Catalog #	Availability	Size / Price	Qty
MAB61462-100
MAB61462-SP

Detection of Human alpha ‑Galactosidase A/GLA by Western Blot.

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Summary Data Examples Preparation and Storage Reconstitution Calculator Background Product Datasheets Related Research Areas

Human alpha -Galactosidase A/GLA Antibody Summary

Species Reactivity

Human

Specificity

Detects human alpha-Galactosidase A/GLA in direct ELISA.

Source

Recombinant Monoclonal Rabbit IgG Clone # 2520B

Purification

Protein A or G purified from hybridoma culture supernatant

Immunogen

Human alpha-Galactosidase A/GLA
Met1-Leu429
Accession # P06280

Formulation

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.

Label

Unconjugated

Applications

Recommended Concentration

Sample

Western Blot

1 µg/mL

A549 human lung carcinoma cell line, MCF‑7 human breast cancer cell line and NCI‑H460 human large cell lung carcinoma cell line.

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.

Scientific Data

Western Blot

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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.

Reconstitution Calculator

Preparation and Storage

Reconstitution

Reconstitute at 0.5 mg/mL in sterile PBS.

Shipping

Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store immediately at the temperature recommended below.

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 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).

References

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.

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