GAA Polyclonal Antibody, Biotin Conjugated
Applications
Reactivity
Predicted Reactivity
| Overview | |
| Catalog # | bs-13254R-Biotin |
| Product Name | GAA Polyclonal Antibody, Biotin Conjugated |
| Applications |
WB
ELISA
IHC-P
IHC-F
|
| Reactivity | Human, Mouse |
| Predicted Reactivity | Rat, Dog, Sheep, Pig, Horse |
| Specifications | |
| Conjugation | Biotin |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Store at -20C for 12 months. |
| Target | |
| Gene ID | 2548 |
| Swiss Prot | P10253 |
| Subcellular location | Cytoplasm, Cell membrane |
| 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. |
| Background | 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 |
| ELISA | 1:500-1000 |
| IHC-P | 1:200-400 |
| IHC-F | 1:100-500 |