Lysosomal Alpha Glucosidase (GAA) Antibody
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Catalogue No: abx026876
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US$326.25
(Size: 80 µl)
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Datasheet SDS
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.
Research Articles on Lysosomal Alpha Glucosidase (GAA)
| Target | Lysosomal Alpha Glucosidase (GAA) |
| Research Area | Enzymes and Kinases, Metabolism |
| Clonality | Polyclonal |
| Reactivity | Human |
| Tested Applications | ELISA, WB, IHC |
| Host | Rabbit |
| Recommended dilutions | WB: 1/1000, IHC-P: 1/500. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user. |
| Conjugation | Unconjugated |
| Immunogen | KLH-conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA. |
| Isotype | IgG |
| Form | Liquid |
| Purification | Purified through a protein A column, followed by peptide affinity purification. |
| Storage | Aliquot and store at 2-8°C for up to 2 weeks, or at -20°C for long term storage. Avoid repeated freeze/thaw cycles. |
| Shelf Life | 12 months. |
| UniProt Primary AC | P10253 (UniProt, ExPASy) |
| UniProt Secondary AC | Q09GN4, Q14351, Q16302, Q8IWE7 |
| UniProt Entry Name | LYAG_HUMAN |
| KEGG | hsa:2548 |
| String | 9606.ENSP00000305692 |
| Molecular Weight | Calculated MW: 105 kDa |
| Buffer | PBS containing 0.09% sodium azide. |
| Availability | Shipped within 5-10 working days. |
| Note | THIS PRODUCT IS FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC, THERAPEUTIC OR COSMETIC PROCEDURES. NOT FOR HUMAN OR ANIMAL CONSUMPTION. |