89-7795-47 Recombinant Human Alpha-Galactosidase A/GLA Protein (His Tag) 50ug PKSH033249
[Elabscience]
特徴
- 背景：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.
仕様
- サイズ：50ug
- 種由来：Human
- 発現宿主：Human Cells
- 純度：> 95 % as determined by reducing SDS-PAGE.
- エンドトキシン濃度：< 1.0 EU per ug as determined by the LAL method.
- アクセッションＮｏ．：P06280
- タグ種類：C-6His
- アミノ酸配列：Leu32-Leu429
- 遺伝子構造：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.
- 別名（遺伝子名）：Alpha-Galactosidase A; Alpha-D-Galactosidase A; Alpha-D-Galactoside Galactohydrolase; Melibiase; Agalsidase; GLA;GLAL
- 分子量：46.4 kDa
- 組成：Supplied as a 0.2 μm filtered solution of 20mM TrisHCl, 150mM NaCl, pH 8.0.
- 保存条件：Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles.
- メーカーマニュアルリンク：https://www.elabscience.com/viewpdf-106911-Elabscience-PKSH033249.pdf
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89-7795-47
Recombinant Human Alpha-Galactosidase A/GLA Protein (His Tag) 50ug
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89-7795-46
Recombinant Human Alpha-Galactosidase A/GLA Protein (His Tag) 10ug
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