Recombinant Human GLA (C-6His)
| Code | Size | Price |
|---|
| ELK-EPT291-1mg | 1mg | £3,368.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Human
Regulatory Status: RUO
Shipping:
The product is shipped on dry ice/polar packs.
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.
Further Information
Accession:
P06280
Alternative Names:
Alpha-Galactosidase A; Alpha-D-Galactosidase A; Alpha-D-Galactoside Galactohydrolase; Melibiase; Agalsidase; GLA
Apparent Molecular Weight:
50-60 KDa, reducing conditions
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.
Biological Activity:
Measured by its ability to hydrolyze 4-Nitrophenyl -alpha -D-galactopyranoside. The specific activity is 2835 pmol/min/ug.
Endotoxin:
Less than 0.1 ng/ug (1 EU/ug) as determined by LAL test.
Molecular Weight:
46.39 KDa
Purity:
Greater than 95% as determined by reducing SDS-PAGE.