Recombinant Human Lysosomal alpha-Glucosidase/GAA, CF

R&D Systems, part of Bio-Techne | Catalog # 8329-GH

R&D Systems, part of Bio-Techne
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8329-GH-025
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Key Product Details

Source

HEK293

Accession #

P10253

Conjugate

Unconjugated

Applications

Enzyme Activity

View all Lysosomal alpha-Glucosidase Proteins and Enzymes »

Product Specifications

Source

Human embryonic kidney cell, HEK293-derived human Lysosomal alpha-Glucosidase protein
Ala70-Cys952, with an N-terminal 6-His tag

Purity

>95%, by SDS-PAGE under reducing conditions and visualized by Colloidal Coomassie® Blue stain at 5 μg per lane.

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

95-105 kDa, reducing conditions

Activity

Measured by its ability to release glucose from starch.
The specific activity is >7,500 pmol/min/μg, as measured under the described conditions.

Reviewed Applications

Read 4 reviews rated 5 using 8329-GH in the following applications:

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

Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein Enzyme Activity Diagram.

Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein, CF (Catalog # 8329-GH) hydrolyses both alpha-1,4- and alpha-1,6-glucosidic linkages on glycogen to release terminal glucose.

Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein Enzyme Activity.

Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein, CF (Catalog # 8329-GH) is measured by its ability to release glucose from starch.

Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein SDS-PAGE.

2 μg/lane of Recombinant Human Lysosomal alpha-Glucosidase/GAA Protein (Catalog # 8329-GH) was resolved with SDS-PAGE under reducing (R) and non-reducing (NR) conditions and visualized by Coomassie® Blue staining, showing bands at 95-105 kDa.

Formulation, Preparation and Storage

8329-GH
Formulation Supplied as a 0.2 μm filtered solution in Tris, NaCl and Glycerol.
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.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 6 months, -20 to -70 °C under sterile conditions after opening.

Background: Lysosomal alpha-Glucosidase

Acid alpha-glucosidase (GAA) is an enzyme that is essential in the degradation of glycogen to glucose in the lysosome (1). Defects in GAA are the cause of glycogen storage disease II, also known as Pompe's disease, which is a rare autosomal recessive metabolic disorder that damages muscle and nerve cells throughout the body, primarily due to the accumulation of glycogen in the lysosome (2). 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 (3). Enzyme replacement therapy (ERT) is used to treat patients with this disease (4, 5).

References

  1. Hoefsloot L.H. et al. (1988) EMBO J. 7:1697.
  2. Wan, L. et al. (2008) J. Neurol. 255:831.
  3. Fukuda, T. et al. (2007) Curr. Neurol. Neurosci. Rep. 7:71.
  4. Van Gelder, C.M. et al. (2014) J Inherit Metab Dis. In press.
  5. Toscano, A. and Schoser, B. (2013) J. Neurol. 260:951.

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

P10253

Additional Lysosomal alpha-Glucosidase Products

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

Product Datasheets

Certificate of Analysis

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

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