Human alpha-Galactosidase A/GLA Antibody (1029734)

R&D Systems | Catalog # MAB61461

R&D Systems

Key Product Details

Species Reactivity

Human

Applications

Immunocytochemistry

Label

Unconjugated

Antibody Source

Monoclonal Mouse IgG1 Clone # 1029734
View all alpha-Galactosidase A/GLA Primary Antibodies »

Product Specifications

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.

Clonality

Monoclonal

Host

Mouse

Isotype

IgG1

Scientific Data Images for Human alpha-Galactosidase A/GLA Antibody (1029734)

alpha ‑Galactosidase A/GLA in A549 Human Cell Line.

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 8 µg/mL for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Mouse IgG Secondary Antibody (red; NL007) and counterstained with DAPI (blue). Specific staining was localized to cytoplasm. Staining was performed using our protocol for Fluorescent ICC Staining of Non-adherent Cells.

Applications for Human alpha-Galactosidase A/GLA Antibody (1029734)

Application
Recommended Usage

Immunocytochemistry

8-25 µg/mL
Sample: Immersion fixed A549 human lung carcinoma cell line

Formulation, Preparation, and Storage

Purification

Protein A or G purified from hybridoma culture supernatant

Reconstitution

Reconstitute at 0.5 mg/mL in sterile PBS. For liquid material, refer to CoA for concentration.

Formulation

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.

Shipping

Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store immediately at the temperature recommended below.

Stability & 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.

Calculators

The reconstitution calculator allows you to quickly calculate the volume of a reagent to reconstitute your vial. Simply enter the mass of reagent and the target concentration and the calculator will determine the rest.

=
÷

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 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).

References

  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.

Alternate Names

Agalsidase alpha, GALA, Melibiase

Entrez Gene IDs

2717 (Human)

Gene Symbol

GLA

UniProt

P06280

Additional alpha-Galactosidase A/GLA Products

Product Documents for Human alpha-Galactosidase A/GLA Antibody (1029734)

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot or batch number in the search box below.

Note: Certificate of Analysis not available for kit components.

Download SDS

Product Specific Notices for Human alpha-Galactosidase A/GLA Antibody (1029734)

For research use only

Customer Reviews for Human alpha-Galactosidase A/GLA Antibody (1029734)

There are currently no reviews for this product. Be the first to review Human alpha-Galactosidase A/GLA Antibody (1029734) and earn rewards!

Have you used Human alpha-Galactosidase A/GLA Antibody (1029734)?

Submit a review and receive an Amazon gift card!

$25/€18/£15/$25CAN/¥2500 for a review with an image

$10/€7/£6/$10CAN/¥1110 for a review without an image

Submit a review
Amazon Gift Card

FAQs

No product specific FAQs exist for this product.

View all FAQs for Antibodies