89-7808-84 Recombinant Mouse alpha-Galactosidase A/GLA Protein (His Tag)(Active) 50ug PKSM040413
[Elabscience]
特徴
- 背景：Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease where 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. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. 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. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.
仕様
- サイズ：50ug
- 種由来：Mouse
- 発現宿主：HEK293 Cells
- 純度：> 95 % as determined by SDS-PAGE
- エンドトキシン濃度：< 1.0 EU per μg of the protein as determined by the LAL method.
- アクセッションＮｏ．：Q8BGZ6
- タグ種類：C-His
- アミノ酸配列：Met1-Arg421
- 遺伝子構造：A DNA sequence encoding the mouse Gla (Q8BGZ6) (Met1-Arg421) was expressed with a C-terminal polyhistidine tag.
- 活性：Measured by its ability to hydrolyze 4-methylumbelliferyl-α-D-galactopyranoside.The specific activity is > 400 pmoles/min/μg.
- 別名（遺伝子名）：Ags
- 分子量：45.6 kDa
- 組成：Lyophilized from sterile PBS, pH 7.4
- 保存条件：Lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months.
- メーカーマニュアルリンク：https://www.elabscience.com/viewpdf-38572-Elabscience-PKSM040413.pdf
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| 商品イメージ | アズワン品番 商品名 |
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89-7808-84
Recombinant Mouse alpha-Galactosidase A/GLA Protein (His Tag)(Active) 50ug
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89-7808-83
Recombinant Mouse alpha-Galactosidase A/GLA Protein (His Tag)(Active) 20ug
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