Anti-AGA Antibody

The Anti-AGA polyclonal antibody from Antibodies.com is raised in rabbit against aspartylglucosaminidase, the lysosomal hydrolase encoded by the AGA gene. This enzyme cleaves N-linked glycopeptides during glycoprotein catabolism in the lysosome, and loss-of-function variants in AGA cause aspartylglucosaminuria, a progressive lysosomal storage disorder characterized by intellectual disability, coarse facial features, skeletal abnormalities, and connective tissue changes. Reagents that detect aspartylglucosaminidase are used to examine enzyme expression, processing, and subcellular trafficking in patient-derived fibroblasts, murine tissue samples, and heterologous overexpression systems.

Provided as an unconjugated IgG preparation in a 200 µl vial, the antibody is validated for Western blot and immunofluorescence workflows. It recognizes the target in both human and murine samples, supporting cross-species comparative work. Typical Western blot protocols use reducing SDS-PAGE with chemiluminescent detection, while immunofluorescence staining often requires fixation conditions chosen to preserve the lysosomal localization that characterizes this hydrolase. Store at -20°C in working aliquots to limit freeze-thaw cycles and preserve binding activity over the shelf life of the preparation.