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Conditions of optimal GLA Enzyme performance should be determined experimentally by the investigator.
Price/availability/specifications subject to change without notice. Unless otherwise indicated, our catalog and customized products are for research use only and not intended for human or animal diagnostic or therapeutic use.
Phone: 1-617-401-8149
Fax: 1-617-606-5019
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Or leave a message with a formal purchase
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Introduction
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.
BP001509-C351: Recombinant Human Alpha-galactosidase A (GLA) Enzyme
Source: HEK 293-derived.
Recombinant Human Alpha-galactosidase A (GLA) Enzyme is a secreted protein with sequence (Leu32 Leu429) of Human GLA fused with a polyhistidine tag at the C-terminus.
Purity: > 95% by SEC-HPLC and reducing SDS-PAGE.
Endotoxin: < 0.1 ng/ug (1 IEU/ug).
Formulation: 0.2 um filtered solution of 20 mM Tris-HCl, 150 mM NaCl, pH 8.0.
Shipping: The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage: Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
12 months from date of receipt, -20 to -70°C as supplied.
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