Key features and details
- Rabbit polyclonal to GAA
- Suitable for: WB
- Reacts with: Mouse, Human
- Isotype: IgG
Product nameAnti-GAA antibody
See all GAA primary antibodies
DescriptionRabbit polyclonal to GAA
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Mouse, Human
Full length protein, corresponding to amino acids 1-952 of Human GAA.
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Storage instructionsShipped at 4°C. Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Storage bufferpH: 7.4
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab102815 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/500 - 1/1000. Predicted molecular weight: 105 kDa.|
FunctionEssential for the degradation of glygogen to glucose in lysosomes.
Involvement in diseaseDefects in GAA are the cause of glycogen storage disease type 2 (GSD2) [MIM:232300]; also called acid alpha-glucosidase (GAA) deficiency or acid maltase deficiency (AMD). GSD2 is a metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy.
Sequence similaritiesBelongs to the glycosyl hydrolase 31 family.
Contains 1 P-type (trefoil) domain.
modificationsThe different forms of acid glucosidase are obtained by proteolytic processing.
Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor.
Cellular localizationLysosome. Lysosome membrane.
- Information by UniProt
- 70 kDa lysosomal alpha-glucosidase antibody
- Acid alpha glucosidase antibody
- Acid maltase antibody
Anti-GAA antibody (ab102815) at 1/500 dilution + Mouse intestine lysate at 50 µg
Predicted band size: 105 kDa
All lanes : Anti-GAA antibody (ab102815) at 1/500 dilution
Lane 1 : GAA transfected 293T cell line
Lane 2 : Non transfected 293T cell line
Lysates/proteins at 25 µg per lane.
Predicted band size: 105 kDa
ab102815 has not yet been referenced specifically in any publications.