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Recombinant Human Alpha-Galactosidase A/GLA Protein (His Tag)

Uniprot : P06280
  • Cat.No.:PKSH033249

  • Expression host: HEK293 Cells

To Purchase PKSH033249

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  • 10μg
  • 50μg
Price: $289
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Description

Synonyms Alpha-Galactosidase A;Alpha-D-Galactosidase A;Alpha-D-Galactoside Galactohydrolase;Melibiase;Agalsidase;GLA;GLAL
Species Human
Expression_host HEK293 Cells
Sequence Leu32-Leu429
Accession P06280
Mol_Mass 46.4 kDa
AP_Mol_Mass 50-60 kDa
Tag C-His

Properties

Purity > 95 % as determined by reducing SDS-PAGE.
Endotoxin level < 1.0 EU per μg of the protein as determined by the LAL method.
Storage Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles.
Shipping This product is provided as liquid. It is shipped at frozen temperature with blue ice/gel packs. Upon receipt, store it immediately at < - 20°C.
Formulation Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.0.
Reconstitution Not Applicable

Background

α-Galactosidase A is a homodimeric glycoprotein that belongs to the glycosyl hydrolase 27 family. It is a lysosomal enzyme and used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. α-Galactosidase A can hydrolyze terminal α-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. Defects α-Galactosidase A are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease with glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease.

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