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Invitrogen™ Human GAA (aa 143-251) Control Fragment Recombinant Protein
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Quantity:
100 μL
Unit Size:
100µL
Description
Highest antigen sequence indentity to the following orthologs: Mouse (84%), Rat (84%). This recombinant protein control fragment may be used for blocking experiments with the corresponding antibody, PA5-82947 (PA5-82947. In IHC/ICC and WB experiments, we recommend a 100x molar excess of the protein fragment control based on the concentration and the molecular weight. Pre-incubate the antibody-protein control fragment mixture for 30 min at room temperature.
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.
Specifications
Specifications
| Accession Number | P10253 |
| Concentration | ≥5.0 mg/mL |
| For Use With (Application) | Blocking Assay, Control |
| Formulation | 1 M urea, PBS with no preservative; pH 7.4 |
| Gene ID (Entrez) | 2548 |
| Name | Human GAA (aa 143-251) Control Fragment |
| Quantity | 100 μL |
| Regulatory Status | RUO |
| Gene Alias | 70 kDa lysosomal alpha-glucosidase; 76 kDa lysosomal alpha-glucosidase; acid (Pompe disease, glycogen storage disease type II); acid alpha-glucosidase; acid maltase; Aglucosidase alfa; E430018M07Rik; Gaa; glucosidase alpha, acid; glucosidase, alpha, acid; glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II); LYAG; Lysosomal alpha-glucosidase |
| Common Name | GAA |
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