Recombinant Human GAA protein, MYC/DDK-tagged
Cat.No. : | GAA-175H |
Product Overview : | Recombinant Human GAA, transcript variant 3, fused with MYC/DDK tag at C-terminal was expressed in HEK293. |
- Specification
- Gene Information
- Related Products
Description : | 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. |
Source : | HEK293 |
Species : | Human |
Tag : | MYC/DDK |
Form : | 25 mM Tris.HCl, pH 7.3, 100 mM glycine, 10% glycerol. |
Molecular Mass : | 102.5 kDa |
Purity : | > 80% as determined by SDS-PAGE and Coomassie blue staining. |
Concentration : | >50 ug/mL as determined by microplate BCA method |
Gene Name : | GAA glucosidase, alpha; acid [ Homo sapiens ] |
Official Symbol : | GAA |
Synonyms : | GAA; glucosidase, alpha; acid; lysosomal alpha-glucosidase; glycogen storage disease type II; Pompe disease; acid maltase; aglucosidase alfa; LYAG; |
Gene ID : | 2548 |
mRNA Refseq : | NM_001079804 |
Protein Refseq : | NP_001073272 |
MIM : | 606800 |
UniProt ID : | P10253 |
Chromosome Location : | 17q25.2-q25.3 |
Pathway : | Galactose metabolism, organism-specific biosystem; Galactose metabolism, conserved biosystem; Lysosome, organism-specific biosystem; Lysosome, conserved biosystem; Metabolic pathways, organism-specific biosystem; Notch-mediated HES/HEY network, organism-specific biosystem; Starch and sucrose metabolism, organism-specific biosystem; |
Function : | alpha-glucosidase activity; carbohydrate binding; hydrolase activity, hydrolyzing O-glycosyl compounds; maltose alpha-glucosidase activity; |
Products Types
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◆ Lysates | ||
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◆ Assay kits | ||
Kit-0353 | GAA Activity Colorimetric Assay Kit | +Inquiry |
Related Gene
For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.
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Q&As (5)
Ask a questionGAA protein is one of several enzymes involved in glycogen metabolism, each playing a specific role in glycogen breakdown.
Enzyme replacement therapy (ERT) and gene therapy are two treatment options available for Pompe disease patients to replace or correct the deficient GAA protein.
GAA protein deficiency is primarily associated with Pompe disease, but there are other glycogen storage diseases related to deficiencies of different enzymes.
ERT involves the infusion of recombinant GAA protein to replace the missing or deficient enzyme in Pompe disease patients.
While GAA protein therapy can improve the symptoms and quality of life for Pompe disease patients, it may not fully reverse the damage caused by the disease.
Customer Reviews (3)
Write a reviewAdditionally, cost-effectiveness, competitive pricing, and bulk purchase options can also be advantageous for researchers working within limited budgets.
The ability to customize the GAA protein variants or modifications offered by the manufacturer is also advantageous, as it allows me to tailor the protein to my specific research objectives.
I appreciate the manufacturer's commitment to maintaining exceptional protein quality standards, which ensures reliable and consistent results in my trials.
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