ANTI-A-GALACTOSIDASE A. CLONE 6G8 (C005B-449038)

General description

a-Galactosidase A (UniProt: P06280; EC:3.2.1.22, Alpha-D-galactosidase A, Alpha-D-galactoside galactohydrolase, Galactosylgalactosylglucosylceramidas GLA, Melibiase, Agalsidase) is encoded by the GLA gene (Gene ID: 2717) in human. a-galactosidase A is a homodimeric, lysosomal enzyme that catalyzes the hydrolysis of terminal alpha-galactosyl moieties from glycolipids and glycoproteins and participates to their degradation in the lysosome. It is synthesized with a signal peptide (aa 1-31), which is subsequently cleaved off to generate the mature form that is heavily glycosylated with asparagine-linked high mannose groups. Mutations in GLA gene have been linked to Fabry disease that is characterized by glycolipid accumulation in various tissues and leads to other complication such as renal failure and cardiac and cerebral complications.

Specificity

Clone 6G8 is a mouse monoclonal antibody that specifically detects human a-Galactosidase A.

Immunogen

Recombinant full-length human a-Galactosidase A (without the signal peptide).

Application

Quality Control Testing

Evaluated by Immunocytochemistry in Acetone fixed HEK 293 cells..

Immunocytochemistry Analysis: A 1:25 dilution of this antibody detected a-Galactosidase A in acetone fixed HEK 293 cells.

Tested Applications

Immunocytochemistry Analysis: A 1:50 dilution from a representative lot detected a-Galactosidase A in HeLa cells.

Immunohistochemistry Applications: A representative lot detected a-Galactosidase A in Immunohistochemistry applications (Tian, W., et. al. (2019). Nat Commun. 10(1):1785).

Immunocytochemistry Analysis: A representative lot detected a-Galactosidase A in acetone fixed HEK293 cells either HEK293 overexpressing GLA (A), HEK293 wild type (WT; B), or HEK293 cells with KO of GLA (C) (Courtesy of Asst. Prof. Zhang Yang, Ph.D., and Assoc. Prof. Ulla Mandel, Ph.D., Copenhagen Center for Glycomics, University of Copenhagen, Denmark).

Western Blotting Analysis: A representative lot detected a-Galactosidase A in lysates from HEK293 cells overexpressing GLA, HEK293 (WT), but not in HEK293 cells with GLA knockout (Courtesy of Asst. Prof. Zhang Yang, Ph.D., and Assoc. Prof. Ulla Mandel, Ph.D., Copenhagen Center for Glycomics, University of Copenhagen, Denmark).

Note: Actual optimal working dilutions must be determined by end user as specimens, and experimental conditions may vary with the end user

Anti-a-galactosidase A, clone 6G8, Cat. No. MABS1935, is a mouse monoclonal antibody that detects -a-galactosidase A and is tested for use in Immunocytochemistry, Immunohistochemistry, and Western Blotting.

Physical form

Purified mouse monoclonal antibody IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.

Storage and Stability

Stable for 1 year at +2°C to +8°C from date of receipt.

Other Notes

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.