Key features and details
- Biotin Rabbit polyclonal to GAA
- Reacts with: Saccharomyces cerevisiae
- Conjugation: Biotin
- Isotype: IgG
Product nameBiotin Anti-GAA antibody
See all GAA primary antibodies
DescriptionBiotin Rabbit polyclonal to GAA
Species reactivityReacts with: Saccharomyces cerevisiae
Full length native protein (purified) corresponding to Saccharomyces cerevisiae GAA.
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Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. Store In the Dark.
Storage bufferpH: 7.20
Constituent: 100% PBS
Concentration information loading...
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
ab182933 has not yet been referenced specifically in any publications.