Human alpha-Galactosidase A/GLA Alexa Fluor™ Plus 488-conjugated Antibody
R&D Systems, part of Bio-Techne | Catalog # AF6146AFP488
Conjugate
Catalog #
Key Product Details
Species Reactivity
Human
Applications
Western Blot, Immunocytochemistry
Label
Alexa Fluor Plus 488 (Excitation = 493 nm, Emission = 518 nm)
Antibody Source
Polyclonal Sheep IgG
Product Specifications
Immunogen
Chinese hamster ovary cell line CHO-derived recombinant human alpha‑Galactosidase A/GLA
Specificity
Detects human alpha‑Galactosidase A/GLA in direct ELISAs.
Clonality
Polyclonal
Host
Sheep
Isotype
IgG
Applications
Application
Recommended Usage
Immunocytochemistry
Optimal dilution of this antibody should be experimentally determined.
Western Blot
Optimal dilution of this antibody should be experimentally determined.
Formulation, Preparation, and Storage
Formulation
Supplied 0.2 mg/mL in a saline solution containing BSA and Sodium Azide.
Shipping
The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage
Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
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
- 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.
Alternate Names
Agalsidase alpha, GALA, Melibiase
Entrez Gene IDs
2717 (Human)
Gene Symbol
GLA
UniProt
Additional alpha-Galactosidase A/GLA Products
Product Specific Notices
This product is provided under an intellectual property license from Life Technologies Corporation. The transfer of this product is conditioned on the buyer using the purchased product solely in research conducted by the buyer, excluding contract research or any fee for service research, and the buyer must not (1) use this product or its components for (a) diagnostic, therapeutic or prophylactic purposes; (b) testing, analysis or screening services, or information in return for compensation on a per-test basis; or (c) manufacturing or quality assurance or quality control, and/or (2) sell or transfer this product or its components for resale, whether or not resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.
For research use only
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