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Recombinant Human α-Galactosidase/GLA (C-6His)
Recombinant Human alpha-Galactosidase is produced by our Mammalian expression system and the target gene encoding Leu32-Leu429 is expressed with a 6His tag at the C-terminus. Bon Opus Cat. #C351

Recombinant Human α-Galactosidase/GLA (C-6His)

SKU: C351-10ug
$123.00Price
Quantity
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Phone: 800-943-6396 ext#1

Email: orders@bonopusbio.com

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Shipping Information

$48 Standard Overnight flat rate for products shipped in room temperature within the United States.

 

Additional $27 Ice Packs shipment fee is required for liquid proteins.

 

Please contact us to order for  international shipments.

Endotoxin
Less than 0.1 ng/ug (1 EU/ug) as determined by LAL test.
Accession Number
P06280
Species
Human
Expression System
Human Cells
Target Sequence
Leu32-Leu429
Tag
C-His
Formulation
Supplied as a 0.2 um filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.0.
Packaging
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 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.
Purity
Greater than 95% as determined by reducing SDS-PAGE.
Alpha-Galactosidase A; Alpha-D-Galactosidase A; Alpha-D-Galactoside Galactohydrolase; Melibiase; Agalsidase; GLA
Alternative Names
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Citation
NA
Activity
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