Recombinant Human Glucosylceramidase/GBA Protein, CF

R&D Systems, part of Bio-Techne | Catalog # 7410-GHB

R&D Systems, part of Bio-Techne
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7410-GHB-01M
7410-GHB-020
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Key Product Details

Source

CHO

Accession #

P04062.3

Conjugate

Unconjugated

Applications

Enzyme Activity

View all Glucosylceramidase/GBA Proteins and Enzymes »

Product Specifications

Source

Chinese Hamster Ovary cell line, CHO-derived human Glucosylceramidase/GBA protein
Ala40-Gln536, with a C-terminal 6-His tag

Purity

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

Endotoxin Level

<1.0 EU per 1 μg of the protein by the LAL method.

N-terminal Sequence Analysis

Ala40

Predicted Molecular Mass

56 kDa

SDS-PAGE

60-75 kDa, reducing conditions

Activity

Measured by its ability to hydrolyze 4-methylumbelliferyl-beta -D-glucopyranoside.
The specific activity is >200 pmol/min/μg, as measured under the described conditions.

Scientific Data Images for Recombinant Human Glucosylceramidase/GBA Protein, CF

Recombinant Human Glucosylceramidase/GBA Protein Enzyme Activity Diagram.

Recombinant Human Glucosylceramidase/GBA Protein, CF (Catalog # 7410-GHB) cleaves the beta-glucosidic linkage of glucosylceramide, an intermediate in glycolipid metabolism.

Recombinant Human Glucosylceramidase/GBA Protein Enzyme Activity.

Recombinant Human Glucosylceramidase/GBA Protein, CF (Catalog # 7410-GHB) is measured by its ability to hydrolyze 4-methylumbelliferyl-beta -D-glucopyranoside.

Recombinant Human Glucosylceramidase/GBA Protein SDS-PAGE.

2 μg/lane of Recombinant Human Glucosylceramidase/GBA Protein (Catalog # 7410-GHB) was resolved with SDS-PAGE under reducing (R) and non-reducing (NR) conditions and visualized by Coomassie® Blue staining, showing bands at 60-75 kDa.

Formulation, Preparation and Storage

7410-GHB
Formulation Supplied as a 0.2 μm filtered solution in Sodium Citrate.
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, -20 to -70 °C as supplied.
  • 3 months, -20 to -70 °C under sterile conditions after opening.

Background: Glucosylceramidase/GBA

Glucosylceramidase is a lysosomal enzyme that cleaves the beta-glucosidic linkage of glucosylceramide (1, 2), an intermediate in glycolipid metabolism. The mature enzyme has 497 amino acids with a molecular weight of 62 kDa (3). Glycosylation occurs at four of five N-glycosylation sites and is essential for the trafficking and activity of the enzyme (4). The enzyme is activated in lysosomes by saposin C, although the mechanism of activation is not well understood (5). Defects in Glucosylceramidase are the cause of Gaucher disease, also known as glucocerebrosidase deficiency (6). Gaucher disease is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Symptoms of Gaucher disease may include enlarged spleen and liver, liver malfunction, skeletal disorders and bone lesions, severe neurologic complications, swelling of lymph nodes, anemia, low blood platelets and yellow fatty deposits on the white of the eye (7). Currently, enzyme replacement therapy is used to treat patients with the disease (8, 9).

References

  1. Sorge, J. et al. (1985) Proc. Natl. Acad. Sci. USA 82:7289.
  2. Ginns, E. I. et al. (1984) Biochem. Biophyl. Res. Commun. 123:574.
  3. Horowitz, M. et al. (1989) Genomics 4:87.
  4. Grace, M.E. et al. (1994) J. Biol. Chem. 269:2283.
  5. Bruhn, h. (2005) Biochem. J. 389:249.
  6. Liou, B. et al. (2006) J. Biol. Chem. 281:4242.
  7. Grabowski, G.A. (2008). Lancet 372:1263.
  8. Zheng, W. et al. (2007) Proc. Natl. Acad. Sci. USA 104:13192.
  9. Beutler, E. and Gelbart, T. (1996) Hum. Mutat. 8:207.

Alternate Names

Alglucerase, GBA, GBA1, Imiglucerase

Entrez Gene IDs

2629 (Human); 14466 (Mouse)

Gene Symbol

GBA

UniProt

P04062.3

Additional Glucosylceramidase/GBA Products

Product Documents for Recombinant Human Glucosylceramidase/GBA Protein, CF

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Product Specific Notices for Recombinant Human Glucosylceramidase/GBA Protein, CF

For research use only

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