Human alpha -Galactosidase A/GLA Antibody

Catalog # Availability Size / Price Qty
MAB6146-100
MAB6146-SP
alpha-Galactosidase A/GLA in Human Testis Tissue.
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Human alpha -Galactosidase A/GLA Antibody Summary

Species Reactivity
Human
Specificity
Detects human alpha-Galactosidase A/GLA in direct ELISAs.
Source
Monoclonal Mouse IgG1 Clone # 1029705
Purification
Protein A or G purified from hybridoma culture supernatant
Immunogen
Chinese Hamster Ovary cell line, CHO-derived human alpha-Galactosidase A/GLA
Met1-Leu429
Accession # P06280
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.
Label
Unconjugated

Applications

Recommended Concentration
Sample
Immunohistochemistry
5-25 µg/mL
Immersion fixed paraffin-embedded sections of human testis tissue

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Scientific Data

Immunohistochemistry View Larger

alpha-Galactosidase A/GLA in Human Testis Tissue. alpha ‑Galactosidase A/GLA was detected in immersion fixed paraffin-embedded sections of human testis tissue using Mouse Anti-Human alpha ‑Galactosidase A/GLA Monoclonal Antibody (Catalog # MAB6146) at 5 µg/mL for 1 hour at room temperature followed by incubation with the Anti-Mouse IgG VisUCyte™ HRP Polymer Antibody (VC001). Before incubation with the primary antibody, tissue was subjected to heat-induced epitope retrieval using Antigen Retrieval Reagent-Basic (CTS013). Tissue was stained using DAB (brown) and counterstained with hematoxylin (blue). Specific staining was localized to Leydig cells. Staining was performed using our IHC Staining with VisUCyte HRP Polymer Detection Reagents protocol.

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Preparation and Storage

Reconstitution
Reconstitute at 0.5 mg/mL in sterile PBS.
Reconstitution Buffer Available
Reconstitution Buffer 1 (PBS)
Catalog #
Availability
Size / Price
Qty
RB01
Shipping
The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
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.

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.
Entrez Gene IDs
2717 (Human)
Alternate Names
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

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