Human alpha-Galactosidase A/GLA Antibody

R&D Systems, part of Bio-Techne | Catalog # MAB61462

Recombinant Monoclonal Antibody.
R&D Systems, part of Bio-Techne
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MAB61462-100
MAB61462-SP
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Key Product Details

Species Reactivity

Human

Applications

Western Blot

Label

Unconjugated

Antibody Source

Recombinant Monoclonal Rabbit IgG Clone # 2520B

View all alpha-Galactosidase A/GLA Antibodies »

Product Specifications

Immunogen

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

Specificity

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

Clonality

Monoclonal

Host

Rabbit

Isotype

IgG

Scientific Data Images for Human alpha-Galactosidase A/GLA Antibody

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.

Applications for Human alpha-Galactosidase A/GLA Antibody

Application
Recommended Usage

Western Blot

1 µg/mL
Sample: A549 human lung carcinoma cell line, MCF-7 human breast cancer cell line and NCI-H460 human large cell lung carcinoma cell line.

Formulation, Preparation, and Storage

Purification

Protein A or G purified from hybridoma culture supernatant

Reconstitution

Reconstitute at 0.5 mg/mL in sterile PBS. For liquid material, refer to CoA for concentration.

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.

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 alpha1‑4Gal beta1‑4Glc 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.

Alternate Names

Agalsidase alpha, GALA, Melibiase

Entrez Gene IDs

2717 (Human)

Gene Symbol

GLA

UniProt

P06280

Additional alpha-Galactosidase A/GLA Products

Product Documents for Human alpha-Galactosidase A/GLA Antibody

Product Datasheets

Certificate of Analysis

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Product Specific Notices for Human alpha-Galactosidase A/GLA Antibody

For research use only

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