Human alpha-Galactosidase A/GLA Antibody

R&D Systems, part of Bio-Techne | Catalog # MAB61461

R&D Systems, part of Bio-Techne
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MAB61461-100
MAB61461-SP
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Key Product Details

Species Reactivity

Validated:

Human

Applications

Immunocytochemistry

Label

Unconjugated

Antibody Source

Monoclonal Mouse IgG1 Clone # 1029734

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Product Summary for Human alpha-Galactosidase A/GLA Antibody

Immunogen

Chinese Hamster Ovary cell line CHO-derived human alpha-Galactosidase A/GLA protein
Met1-Leu429
Accession # P06280

Specificity

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

Clonality

Monoclonal

Host

Mouse

Isotype

IgG1

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

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.

Applications for Human alpha-Galactosidase A/GLA Antibody

Application
Recommended Usage

Immunocytochemistry

8-25 µg/mL
Sample: Immersion fixed A549 human lung carcinoma cell line
Please Note: Optimal dilutions of this antibody should be experimentally determined.

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.

Reconstitution Buffer Available:
Size / Price
Qty

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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