Recombinant Human Alpha-Galactosidase A/GLA Protein (His Tag)

Cat. No : CDRP-00329

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Target Information
Target Name	Alpha-Galactosidase A/GLA
Synonyms	Alpha-Galactosidase A; Alpha-D-Galactosidase A; Alpha-D-Galactoside Galactohydrolase; Melibiase; Agalsidase; GLA; GLAL
UniProt ID	P08253
Accession	P06280

Product Details
Expression Host	HEK293 Cells
Species	Human
Tag	C-6His
Molar Mass	46.4 kDa
Sequence	Ser 607-Pro 1265

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.

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.

Properties
Purity	>97% as determined by reducing SDS-PAGE.
Formulation	Supplied as a 0.2 μm filtered solution of 20 mM Tris-HCl, 150 mM NaCl, pH8.0.
Reconstitution	Please refer to the printed manual for detailed information.
Shipping	This product is provided as liquid. It is shipped at frozen temperature with blue ice/gel packs.
Storage	Store at < -20 °C, stable for 6 months. Please minimize freeze-thaw cycles.

! For research use only. Not intended for any clinical use.