Human alpha -Galactosidase A/GLA Antibody

Name: Human alpha -Galactosidase A/GLA Antibody
Brand: R&D Systems
SKU: MAB61461

R&D Systems, Inc. a Bio-Techne Brand

Catalog # MAB61461

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Key Product Details

Species Reactivity:	Human
Immunogen:	Chinese Hamster Ovary cell line CHO-derived human alpha-Galactosidase A/GLA protein Met1-Leu429 Accession # P06280
Applications:	Immunocytochemistry/Immunofluorescence
Label:	Unconjugated
Source:	Monoclonal Mouse IgG₁ Clone # 1029734
Specificity:	Detects human alpha-Galactosidase A/GLA in direct ELISAs.

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Applications for Human alpha -Galactosidase A/GLA Antibody

	Recommended Concentration	Sample
Immunocytochemistry	8-25 µg/mL	Immersion fixed A549 human lung carcinoma cell line

Please Note: Optimal dilutions should be determined by each laboratory for each application.

Scientific Data Examples for Human alpha -Galactosidase A/GLA Antibody

	Immunocytochemistry 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.

Formulation, Preparation and Storage

Purification	Protein A or G purified from hybridoma culture supernatant
Reconstitution	Reconstitute at 0.5 mg/mL in sterile PBS. Reconstitution Buffer Available: Size / Price Qty Loading... RB01:
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	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.

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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).

Item	Value
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, Agalsidase alpha, Alpha-D-galactosidase A, Alpha-D-galactoside galactohydrolase, EC 3.2.1, EC 3.2.1.22, GALA, GLA, Melibiase, agalsidase alfa, alpha-D-galactoside galactohydrolase 1, alpha-gal A, alpha-galactosidase A, galactosidase, alpha

Citations for Human alpha -Galactosidase A/GLA Antibody

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Human alpha -Galactosidase A/GLA Antibody

R&D Systems, Inc. a Bio-Techne Brand

Key Product Details

Complete Your Research

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Applications for Human alpha -Galactosidase A/GLA Antibody

Scientific Data Examples for Human alpha -Galactosidase A/GLA Antibody

Immunocytochemistry

Formulation, Preparation and Storage

Reconstitution Calculator

Background: alpha-Galactosidase A/GLA

Citations for Human alpha -Galactosidase A/GLA Antibody

Product Documents for Human alpha -Galactosidase A/GLA Antibody

Product Datasheets

COA

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