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Recombinant Human alpha-Galactosidase A/GLA Protein, CF

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Recombinant Human alpha-Galactosidase A/GLA Protein, CF (Catalog # 6146-GH) hydrolyses the terminal alpha -galactosyl moieties from glycolipids and glycoproteins predominantly ceramide trihexoside.
Recombinant Human alpha-Galactosidase A/GLA Protein, CF (Catalog # 6146-GH) hydrolyses the terminal alpha -galactosyl moieties from glycolipids and glycoproteins predominantly ceramide trihexoside.
Recombinant Human alpha-Galactosidase A/GLA Protein, CF (Catalog # 6146-GH) is measured by its ability to hydrolyze 4-methylumbelliferyl-alpha -D-galactopyranoside.
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, ...read more

Product Details

Summary
Reactivity HuSpecies Glossary
Applications Enzyme Activity
Format
Carrier-Free

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Recombinant Human alpha-Galactosidase A/GLA Protein, CF Summary

Details of Functionality
Measured by its ability to hydrolyze 4-methylumbelliferyl-alpha -D-galactopyranoside.<br />The specific activity is &gt;1,100 pmol/min/μg, as measured under the described conditions. <br /><br />
Accession #
N-terminal Sequence
Leu32
Protein/Peptide Type
Recombinant Enzymes
Gene
GLA
Endotoxin Note
<1.000 EU per 1 µg of the protein by the LAL method.

Applications/Dilutions

Dilutions
  • Enzyme Activity
Theoretical MW
46 kDa.
Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.
SDS-PAGE
45-55 kDa, reducing conditions
Publications
Read Publications using
6146-GH in the following applications:

Packaging, Storage & Formulations

Storage
Store the unopened product at -70 °C. Use a manual defrost freezer and avoid repeated freeze-thaw cycles. Do not use past expiration date.
Buffer
Supplied as a 0.2 μm filtered solution in Tris and NaCl.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for Recombinant Human alpha-Galactosidase A/GLA Protein, CF

  • agalsidase alfa
  • Agalsidase alpha
  • Agalsidase
  • Alpha-D-galactosidase A
  • alpha-D-galactoside galactohydrolase 1
  • Alpha-D-galactoside galactohydrolase
  • alpha-gal A
  • alpha-galactosidase A
  • EC 3.2.1
  • EC 3.2.1.22
  • GALA
  • galactosidase, alpha
  • GLA
  • Melibiase

Background

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 alpha 1‑4Gal beta 1‑4Glc 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).

  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.

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Recombinant Human alpha-Galactosidase A/GLA Protein, CF
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Publications for alpha-Galactosidase A/GLA (6146-GH)(3)

We have publications tested in 2 confirmed species: Human, N/A.

We have publications tested in 2 applications: Bioassay, Western Blot Control.


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Bioassay
(2)
Western Blot Control
(1)
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Human
(2)
N/A
(1)
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Bioinformatics

Gene Symbol GLA
Uniprot