ARG58730
anti-GAA antibody
anti-GAA antibody for ICC/IF,Western blot and Human,Mouse,Rat
Overview
| Product Description | Rabbit Polyclonal antibody recognizes GAA |
|---|---|
| Tested Reactivity | Hu, Ms, Rat |
| Tested Application | ICC/IF, WB |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | IgG |
| Target Name | GAA |
| Antigen Species | Human |
| Immunogen | Synthetic peptide within aa. 350-450 of Human GAA (NP_000143.2). |
| Conjugation | Un-conjugated |
| Alternate Names | Lysosomal alpha-glucosidase; LYAG; Aglucosidase alfa; Acid maltase; EC 3.2.1.20 |
Application Instructions
| Application Suggestion |
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| Application Note | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. | ||||||
| Positive Control | Mouse thymus | ||||||
| Observed Size | 105 kDa |
Properties
| Form | Liquid |
|---|---|
| Purification | Affinity purified. |
| Buffer | PBS (pH 7.3), 0.02% Sodium azide and 50% Glycerol. |
| Preservative | 0.02% Sodium azide |
| Stabilizer | 50% Glycerol |
| Storage Instruction | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
| Note | For laboratory research only, not for drug, diagnostic or other use. |
Bioinformation
| Database Links | |
|---|---|
| Gene Symbol | GAA |
| Gene Full Name | glucosidase, alpha; acid |
| 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] |
| Function | Essential for the degradation of glygogen to glucose in lysosomes. [UniProt] |
| Cellular Localization | Lysosome, Lysosome membrane. [UniProt] |
| Calculated MW | 105 kDa |
| PTM | The different forms of acid glucosidase are obtained by proteolytic processing. Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor. [UniProt] |
