| Overview |
| bs-13254R-PE |
| Alpha Glucosidase Polyclonal Antibody, PE Conjugated |
| WB |
| Human, Mouse, Rat |
| Dog, Sheep, Pig, Horse |
| Specifications |
| PE |
| Rabbit |
| KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha |
| 541-640/952 |
| Polyclonal |
| IgG |
| 1ug/ul |
| Purified by Protein A. |
| Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
| Target |
| 2548 |
| P10253 |
| Cytoplasm, Cell membrane |
| 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. |
| 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. [provided by RefSeq, Jul 2008]. |
| Application Dilution |
| WB |
1:300-5000 |
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