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The antibody against GAA was raised in Rabbit using a synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IF/ICC, ELISA.
The antibody against GAA was raised in Rabbit using a synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IF/ICC, ELISA.
| Cat.No | ADA-09295A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | GAA |
| Target Synonyms | LYAG; GAA | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.01% thimerosal, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Rat brain, DU145, Mouse brain, Mouse liver, Mouse ovary | Application | ELISA, WB, IF/ICC |
| Immunogen Description | A synthetic peptide corresponding to a sequence within amino acids 350-450 of human GAA (NP_000143.2). | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | VVQQYLDVVGYPFMPPYWGLGFHLCRWGYSSTAITRQVVENMTRAHFPLDVQWNDLDYMDSRRDFTFNKDGFRDFPAMVQELHQGGRRYMMIVDPAISSSG | 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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