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alpha-Galactosidase A/GLA Antibody (1029734) [Unconjugated]

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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 ...read more

Product Details

Summary
Reactivity HuSpecies Glossary
Applications ICC/IF
Clone
1029734
Clonality
Monoclonal
Host
Mouse
Conjugate
Unconjugated

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Catalog# & Formulation Size Price
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alpha-Galactosidase A/GLA Antibody (1029734) [Unconjugated] Summary

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.
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
  • Immunocytochemistry 8-25 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 (1029734) [Unconjugated]

  • 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).

  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.

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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