BackgroundThis gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants.
DescriptionGAA Monoclonal Antibody. Unconjugated. Raised in: Rabbit.
FormulationBuffer: PBS containing 50% glycerol and 0.05% BSA, preserved with proclin300 or sodium azide, pH 7.3.
SpecificityHuman
IsotypeIgG
Uniprot IDP10253
PurificationAffinity Purified
ImmunogenSynthetic peptide. Please contact EpigenTek for more information.
StorageStore at -20°C. Avoid freeze / thaw cycles.
Alternative NamesLYAG; GAA
ApplicationWB, ELISA; Recommended dilution: WB, 1:1000 - 1:6000; ELISA, Recommended starting concentration is 1 µg/mL. Please optimize the concentration based on your specific assay requirements.