Recombinant Mouse Lysosomal alpha-Glucosidase/GAA, CF

R&D Systems, part of Bio-Techne | Catalog # 11400-GH

His-tag
R&D Systems, part of Bio-Techne
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11400-GH-025
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Key Product Details

Source

CHO

Accession #

P70699.2

Conjugate

Unconjugated

Applications

Enzyme Activity

View all Lysosomal alpha-Glucosidase Proteins and Enzymes »

Product Specifications

Source

Chinese Hamster Ovary cell line, CHO-derived mouse Lysosomal alpha-Glucosidase protein
Glu70-Ser953 with a N-terminal 6-His tag

Purity

>95%, by SDS-PAGE visualized with Silver Staining and quantitative densitometry by Coomassie® Blue Staining.

Endotoxin Level

<0.10 EU per 1 μg of the protein by the LAL method.

N-terminal Sequence Analysis

His

Predicted Molecular Mass

99 kDa

SDS-PAGE

97-107 kDa, under reducing conditions

Activity

Measured by its ability to release glucose from starch.
The specific activity is >5000 pmol/min/μg, as measured under the described conditions.

Scientific Data Images for Recombinant Mouse Lysosomal alpha-Glucosidase/GAA, CF

Recombinant Mouse Lysosomal alpha-Glucosidase His-tag Protein Enzyme Activity Diagram.

Recombinant Mouse Lysosomal alpha-Glucosidase His-tag Protein, CF (Catalog # 11400-GH) hydrolyses both alpha-1,4- and alpha-1,6-glucosidic linkages on glycogen to release terminal glucose.

Recombinant Mouse Lysosomal alpha‑Glucosidase His-tag Protein SDS-PAGE.

2 μg/lane of Recombinant Mouse Lysosomal alpha‑Glucosidase His-tag Protein (Catalog # 11400-GH) was resolved with SDS-PAGE under reducing (R) and non-reducing (NR) conditions and visualized by Coomassie® Blue staining, showing bands at 97-107 kDa, under reducing conditions.

Formulation, Preparation and Storage

11400-GH
Formulation Supplied as a 0.2 μm filtered solution in Tris and NaCl. 
Shipping The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 6 months from date of receipt, -70 °C as supplied.
  • 3 months, -70 °C under sterile conditions after opening.

Background: Lysosomal alpha-Glucosidase

Acid alpha-glucosidase (GAA) is an essential enzyme for the hydrolysis of glycogen alpha1-4 and alpha1,6-glycosidic linkages within the lysosome (1,2). GAA is a member of the glycoside hydrolase family GH31 and contains an N-terminal trefoil-P domain, a beta-sheet domain, a catalytic barrel, and two C-terminal beta-sheet domains (2). In addition to an active site and substrate binding domain, GAA has an additional reported secondary substrate-binding domain that may enhance the processivity of the enzyme (2). Mouse GAA has approximately 80% homology with human GAA. Defects in GAA cause glycogen storage disease II, also known as Pompe's disease, which is a rare autosomal recessive metabolic disorder that damages muscle and nerve cells due to accumulation of glycogen in the lysosome (3). Pompe disease occurs in babies, children, and adults who inherit a defective GAA gene and affects an estimated 5,000 to 10,000 people worldwide (4). Enzyme replacement therapy (ERT) is used to treat patients with Pompe disease and other lysosomal storage diseases (LSDs) (5, 6). Alternative therapeutic strategies such as pharmacological chaperone therapy (PCT) are being explored for use in concert with or independently for the potential to stabilize the target enzyme without impact to the catalytic activity (2, 7, 8).

References

  1. Hoefsloot, L.H. et. al. (1988) EMBO J. 7:1697.
  2. Roig-Zamboni, V. et. al. (2017) Nat. Commun. 8:1111.
  3. Wan, L. et. al. (2008) J. Neurol. 255:831.
  4. Fukuda, T. et. al. (2007) Curr. Neurol. Neurosci. Rep. 7:71.
  5. Van Gelder, C.M. et. al. (2014) J. Inherit. Metab. Dis. In press.
  6. Toscano, A. and B. Schoser. (2013) J. Neurol. 260:951.
  7. Porto, C. et. al. (2012) Mol. Ther. 20:2201.
  8. Parenti, G. et. al. (2015). Mol. Ther. 23:1138.

Long Name

Glucosidase, Alpha; Acid

Alternate Names

Acid alpha-Glucosidase, Acid Maltase, GAA, LYAG, Lysosomal alphaGlucosidase

Entrez Gene IDs

2548 (Human); 14387 (Mouse); 367562 (Rat); 102141245 (Cynomolgus Monkey)

Gene Symbol

GAA

UniProt

P70699.2

Additional Lysosomal alpha-Glucosidase Products

Product Documents for Recombinant Mouse Lysosomal alpha-Glucosidase/GAA, CF

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Product Specific Notices for Recombinant Mouse Lysosomal alpha-Glucosidase/GAA, CF

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