Recombinant Human alpha-Galactosidase A/GLA Protein, CF

R&D Systems, part of Bio-Techne | Catalog # 6146-GH

R&D Systems, part of Bio-Techne
Catalog #
Availability
Size / Price
Qty
6146-GH-020
Print Quote
Bulk Order
100% Guarantee

Key Product Details

Source

CHO

Accession #

P06280

Conjugate

Unconjugated

Applications

Enzyme Activity

View all alpha-Galactosidase A/GLA Proteins and Enzymes »

Product Specifications

Source

Chinese Hamster Ovary cell line, CHO-derived human alpha-Galactosidase A/GLA protein
Met1-Leu429, 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

Leu32

Predicted Molecular Mass

46 kDa

SDS-PAGE

45-55 kDa, reducing conditions

Activity

Measured by its ability to hydrolyze 4-methylumbelliferyl-alpha -D-galactopyranoside.
The specific activity is >1,100 pmol/min/μg, as measured under the described conditions.

Reviewed Applications

Read 2 reviews rated 5 using 6146-GH in the following applications:

Scientific Data Images for Recombinant Human alpha-Galactosidase A/GLA Protein, CF

Recombinant Human alpha-Galactosidase A/GLA Protein Enzyme Activity Diagram.

Recombinant Human alpha-Galactosidase A/GLA Protein, CF (Catalog # 6146-GH) hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. The common ceramide trihexoside substrate is displayed in the diagram.

Recombinant Human alpha-Galactosidase A/GLA Protein Enzyme Activity.

Recombinant Human alpha-Galactosidase A/GLA Protein, CF (Catalog # 6146-GH) is measured by its ability to hydrolyze 4-methylumbelliferyl-alpha -D-galactopyranoside.

Recombinant Human alpha‑Galactosidase A/GLA Protein SDS-PAGE.

2 μg/lane of Recombinant Human alpha‑Galactosidase A/GLA Protein (Catalog # 6146-GH) was resolved with SDS-PAGE under reducing (R) and non-reducing (NR) conditions and visualized by Coomassie® Blue staining, showing bands at 45-55 kDa.

Formulation, Preparation and Storage

6146-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, -20 to -70 °C as supplied.
  • 3 months, -20 to -70 °C under sterile conditions after opening.

Background: alpha-Galactosidase A/GLA

Human alpha-Galactosidase A is a homodimeric glycoprotein that can release terminal alpha-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose (1). It is a lysosomal enzyme and is responsible for degradation of glycolipid globotriaosylceramide (Gb3) (Gal alpha1‑4Gal beta1‑4Glc beta‑ceramide). Mutations in this gene cause Fabry disease, an X-linked hereditary lysosomal storage disease with the accumulation of Gb3 in the walls of small blood vessels, nerves, dorsal root ganglia, renal glomerular and tubular epithelial cells, and cardiomyocytes (2, 3). Inability to prevent the glycosphingolipid deposition can cause hypertension, strokes, heart attack and progressive renal failure (4). Current treatment for Fabry disease is enzyme replacement therapy using intravenously delivered recombinant alpha-Galactosidase A (5, 6).

References

  1. Ioannou, Y.A. et al. (1998) Biochem. J. 332:789.
  2. Koide, T. et al. (1990) FEBS Lett. 259:353.
  3. Ioannou Y.A, et al. (1992) J. Cell Biol. 119:1137.
  4. Germain, D.P. (2002) Expert. Opin. Investig. Drugs. 11:1467.
  5. Barngrover, D. (2003) J. Biotechnol. 95:280.
  6. Mignani, R. and Cagnoli, L. (2004) J. Nephrol. 17:354.

Alternate Names

Agalsidase alpha, GALA, Melibiase

Entrez Gene IDs

2717 (Human)

Gene Symbol

GLA

UniProt

P06280

Additional alpha-Galactosidase A/GLA Products

Product Documents for Recombinant Human alpha-Galactosidase A/GLA Protein, CF

Product Datasheets

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot number in the search box below.

Note: Certificate of Analysis not available for kit components.

Download SDS

Product Specific Notices for Recombinant Human alpha-Galactosidase A/GLA Protein, CF

For research use only

Privacy and Cookie Policy
Site Map
© Copyright 2024 Bio-Techne. All Rights Reserved.