GAA Recombinant Antibody
Applications
Reactivity
| Overview | |
| Catalog # | bsm-54735r |
| Product Name | GAA Recombinant Antibody |
| Applications |
WB
FCM
IHC-P
|
| Reactivity | Human |
| Specifications | |
| Conjugation | Unconjugated |
| Host | Rabbit |
| Source | Synthetic peptide within Human GAA. |
| Clonality | Monoclonal |
| Clone # | 5H49 |
| Isotype | IgG |
| Concentration | Lot dependent |
| Purification | Purified by Protein A. |
| Storage Buffer | 0.01M TBS(pH7.4) 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 | Lysosome, Lysosome membrane |
| Synonyms | 70 kDa lysosomal alpha-glucosidase antibody, Acid alpha glucosidase antibody, Acid maltase antibody, Aglucosidase alfa antibody, Alpha glucosidase antibody, GAA antibody, Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody, Glucosidase alpha acid antibody, Glucosidase alpha antibody, LYAG antibody, LYAG_HUMAN antibody, Lysosomal alpha glucosidase antibody |
| 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 |
| FCM | 1:20-100 |
| IHC-P | 1:200-400 |