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The recombinant antibody against GAA was produced using a synthesized peptide derived from Human GAA as the immunogen. This antibody exists as a non-conjugated isotype Rabbit IgG, Affinity-chromatography purified. This antibody has been validated on ELISA, IHC.
The recombinant antibody against GAA was produced using a synthesized peptide derived from Human GAA as the immunogen. This antibody exists as a non-conjugated isotype Rabbit IgG, Affinity-chromatography purified. This antibody has been validated on ELISA, IHC.
$350.00
| Cat.No | ADC-56572A | Clonality | Monoclonal |
|---|---|---|---|
| Target Name | GAA | Target Synonyms | 70 kDa lysosomal alpha-glucosidase], GAA, Lysosomal alpha-glucosidase (EC 3.2.1.20) (Acid maltase) (Aglucosidase alfa) [Cleaved into: 76 kDa lysosomal alpha-glucosidase |
| Form | Liquid | Species Reactivity | Human |
| Isotype | Rabbit IgG | Storage Buffer | PH 7.4, 0.02% sodium azide and 50% glycerol., 150mM NaCl, Rabbit IgG in phosphate buffered saline |
| Purification Method | Affinity-chromatography purified | Conjugate | Non-conjugated |
| Application | ELISA, IHC | Storage | Upon receipt |
| Immunogen Description | A synthesized peptide derived from Human GAA | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | Complete sequences for the immunogen, target protein, and peptides are available upon request. | Uniprot ID | P10253 |
Uniprot Id
P10253
Target Species
Human
Target Name
GAA
Target Full Name
Lysosomal alpha-glucosidase
Target Function
Essential for the degradation of glycogen in lysosomes. Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans.
Target Involvement
Glycogen storage disease 2 (GSD2)
Target Subcellular Location
Lysosome. Lysosome membrane.
Target Protein Families
Glycosyl hydrolase 31 family
Target Synonyms
70 kDa lysosomal alpha-glucosidase; Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; Alpha glucosidase; GAA; Glucosidase alpha acid (Pompe disease glycogen storage disease type II); Glucosidase alpha acid; Glucosidase alpha; LYAG; LYAG_HUMAN; Lysosomal alpha glucosidase
Target Background
This 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.
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