alpha-Galactosidase A/GLA Antibody [Alexa Fluor® 350]

• Reactivity: Hu
• Applications: WB, ICC/IF
• Clonality: Polyclonal
• Host: Sheep
• Conjugate: Alexa Fluor 350

alpha-Galactosidase A/GLA Antibody [Alexa Fluor® 350] Summary

• Immunogen: Chinese hamster ovary cell line CHO-derived recombinant human α‑Galactosidase A/GLA
  Leu32-Leu429
  Accession # P06280
• Specificity: Detects human alpha ‑Galactosidase A/GLA in direct ELISAs.
• 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 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide

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 [Alexa Fluor® 350]

• 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.