Specifications
- Pk:50 µG
- Enzyme type:Recombinant
- Source:Cells
- Species:Human
- Environmentally Preferable:
- Tag sequence:C-6 His tag
- Storage conditions:Store at −20 °C, stable for 6 months after receipt. Please minimize freeze−thaw cycles.
- Enzyme name:alpha-Galactosidase A
- Purity:>95% as determined by reducing SDS-PAGE
- Molecular weight:46.39 kD
- Sequence:Leu32-Leu429
- Formulation:Supplied as a 0.2 µm filtered solution of 20 mM TrisHCl, 150 mM NaCl, pH 8.0. Always centrifuge tubes before opening. Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100 ug/ml. Dissolve the lyophilized protein in ddH2O. Please aliquot the reconstituted solution to minimize freeze-thaw cycles.
- Tested applications:Bioassay
Specifications
About this item
alpha-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. alpha-Galactosidase A can hydrolyze terminal alpha-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. Defects alpha-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.
Fusion-Tag: C-6 His tag
This recombinant protein can be used for biological assays. For research use only.