alpha-Galactosidase A/GLA Antibody (1029705) [Unconjugated] Summary
Immunogen |
Chinese Hamster Ovary cell line, CHO-derived human alpha-Galactosidase A/GLA Met1-Leu429 Accession # P06280 |
Specificity |
Detects human alpha-Galactosidase A/GLA in direct ELISAs. |
Source |
N/A |
Isotype |
IgG1 |
Clonality |
Monoclonal |
Host |
Mouse |
Purity Statement |
Protein A or G purified from hybridoma culture supernatant |
Innovator's Reward |
Test in a species/application not listed above to receive a full credit towards a future purchase. |
Applications/Dilutions
Dilutions |
- Immunohistochemistry 5-25 ug/mL
|
Packaging, Storage & Formulations
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.
|
Buffer |
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. |
Reconstitution Instructions |
Reconstitute at 0.5 mg/mL in sterile PBS. |
Notes
This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.
Alternate Names for alpha-Galactosidase A/GLA Antibody (1029705) [Unconjugated]
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).
-
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.
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.
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