Description
| Reference | ESCIT291 |
|---|---|
| Size | 1mg |
| Molecular Weight | 46.39 KDa |
| Purity | >95% by SDS-PAGE. |
| Endotoxin | <1 EU/µg |
| Biological Activity | Measured by its ability to hydrolyze 4-Nitrophenyl -alpha -D-galactopyranoside. The specific activity is 2835 pmol/min/µg. |
Other names: Alpha-Galactosidase A; Alpha-D-Galactosidase A; Alpha-D-Galactoside Galactohydrolase; Melibiase; Agalsidase; GLA
Redissolve:
Storage: Store at ≤-70°C, stable for 6 months after receipt.
Store at ≤-70°C, stable for 3 months under sterile conditions after opening.
Please minimize freeze-thaw cycles.
Shipping Condition: Dry ice/polar packs.
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 rESCIlacement 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 dESCIosits, febrile ESCIisodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease.