alpha-Galactosidase A/GLA Antibody [Alexa Fluor® 532]

• Reactivity: Hu
• Applications: WB, ICC/IF
• Clonality: Polyclonal
• Host: Sheep
• Conjugate: Alexa Fluor 532

alpha-Galactosidase A/GLA Antibody [Alexa Fluor® 532] Summary

• Immunogen: Chinese hamster ovary cell line CHO-derived recombinant human alpha ‑Galactosidase A/GLA
  Leu32-Leu429
  Accession # P06280
• Specificity: Detects human alpha ‑Galactosidase A/GLA in direct ELISAs.
• Source: N/A
• Isotype: IgG
• Clonality: Polyclonal
• Host: Sheep
• Purity Statement: Antigen Affinity-purified

Applications/Dilutions

• Dilutions:
  • Immunocytochemistry
  • Western Blot

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2 mg/mL in a saline solution containing BSA and Sodium Azide.

Notes

This product is provided under an agreement between Life Technologies Corporation and R&D Systems, Inc, and the manufacture, use, sale or import of this product is subject to one or more US patents and corresponding non-US equivalents, owned by Life Technologies Corporation and its affiliates. The purchase of this product conveys to the buyer the non-transferable right to use the purchased amount of the product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). The sale of this product is expressly conditioned on the buyer not using the product or its components (1) in manufacturing; (2) to provide a service, information, or data to an unaffiliated third party for payment; (3) for therapeutic, diagnostic or prophylactic purposes; (4) to resell, sell, or otherwise transfer this product or its components to any third party, or for any other commercial purpose. Life Technologies Corporation will not assert a claim against the buyer of the infringement of the above patents based on the manufacture, use or sale of a commercial product developed in research by the buyer in which this product or its components was employed, provided that neither this product nor any of its components was used in the manufacture of such product. For information on purchasing a license to this product for purposes other than research, contact Life Technologies Corporation, Cell Analysis Business Unit, Business Development, 29851 Willow Creek Road, Eugene, OR 97402, Tel: (541) 465-8300. Fax: (541) 335-0354.

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for alpha-Galactosidase A/GLA Antibody [Alexa Fluor® 532]

• 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

Background

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).

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Bioinformatics

• Uniprot:
  • P06280()