Recombinant Human alpha-Galactosidase A/GLA Protein
别名
重组人alpha-Galactosidase A/GLA蛋白 | 重组人α-半乳糖苷酶A
英文别名
agalsidase alfa | Agalsidase alpha | Agalsidase | Alpha-D-galactosidase A | alpha-D-galactoside galactohydrolase 1 | Alpha-D-galactoside galactohydrolase | alpha-gal A | alpha-galactosidase A | EC 3.2.1 | EC 3.2.1.22 | GALA | galactosidase | alpha | GLA |
规格或纯度
无动物源,无载体,His标签,PBS Only,≥90%(SDS-PAGE),见COA
生化机理
Human alpha-Galactosidase A is a homodimeric glycoprotein that can release terminal alpha-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. It is a lysosomal enzyme and is responsible for degradation of glycolipid globotriaosylceramide (Gb3) (Gal alpha 1‑4Gal beta 1‑4Glc beta ‑ceramide). Mutations in this gene cause Fabry disease, an X-linked hereditary lysosomal storage disease with the accumulation of Gb3 in the walls of small blood vessels, nerves, dorsal root ganglia, renal glomerular and tubular epithelial cells, and cardiomyocytes. Inability to prevent the glycosphingolipid deposition can cause hypertension, strokes, heart attack and progressive renal failure. Current treatment for Fabry disease is enzyme replacement therapy using intravenously delivered recombinant alpha-Galactosidase A.
45.0-55.0 kDa, under reducing conditions; 45.0-55.0 kDa, under non-reducing conditions.
分子类型
蛋白质
产品线
生物产品
储存与运输
物理形态
冻干(Lyophilized)
储存缓冲液
Lyophilized from PBS, pH 7.4
浓度
见COA
复溶
We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Reconstitute at 0.5 mg/mL in sterile distilled water. Stock solutions should be apportioned into working aliquots and stored at ≤ -20 °C. Further dilutions should be made in appropriate buffered solutions.
储存条件
-20°C储存,避免反复冻融
运输条件
超低温运输
稳定性与储存
-20℃保存 12个月；复溶后建议分装，避免反复冻融。
图片
Recombinant Human alpha-Galactosidase A/GLA Protein (rp181244) - SDS-PAGE 3 μg/lane of Recombinant Human alpha-Galactosidase A/GLA Protein was resolved with SDS-PAGE under reducing (R) and non-reducing (N) conditions and visualized by Coomassie® Blue staining, showing a band at 45.0-55.0 kDa.
ZJ25F0826050
危险品化学品经营许可证（带存储）