Recombinant Human Alpha-Galactosidase A/GLA Protein (His Tag)
| Code | Size | Price |
|---|
| PKSH033249-10ug | 10ug | £309.00 |
Quantity:
| PKSH033249-50ug | 50ug | £531.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Human
Regulatory Status: RUO
Shipping:
The product is shipped at ambient temperature. Upon receipt store it immediately at the storage temperature listed.
Storage:
Samples are stable for up to twelve months from date of receipt at -70°C. Store it under sterile conditions at -20°C to -80°C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
Further Information
Abbreviation:
Alpha-Galactosidase A;GLA
Accession:
P06280
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.
Calculated MW:
46.4 kDa
Endotoxin:
< 1.0 EU per ug of the protein as determined by the LAL method.
Expression Host:
HEK293 Cells
Formulation:
Supplied as a 0.2 um filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.0.
Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization.
Please refer to the specific buffer information in the printed manual.
Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization.
Please refer to the specific buffer information in the printed manual.
Fusion tag:
C-6His
ObservedMW:
51 kDa
Purity:
> 95 % as determined by reducing SDS-PAGE.
Sequence:
Leu32-Leu429
Target Synonym:
Alpha-Galactosidase A;Alpha-D-Galactosidase A;Alpha-D-Galactoside Galactohydrolase;Melibiase;Agalsidase;GLA;GLAL
UNIProt ID:
P06280