Human alpha -Galactosidase A/GLA Antibody

Catalog #: MAB6146 Datasheet / COA / SDS

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

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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.

Reconstitution Calculator

Preparation and Storage

Reconstitution

Reconstitute at 0.5 mg/mL in sterile 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.

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 storagedisease with the accumulation of Gb3 in the walls of small blood vessels,nerves, dorsal root ganglia, renal glomerular and tubular epithelialcells, 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

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.

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