R&D Systems代理6415-GH-020 Recombinant E. coli alpha-Galactosidase/a-Gal Protein, CF (20 UG)

2025-06-28

货号:6415-GH-020

品牌:R&D Systems

规格:20ug

目录价:¥4490.00

市场价格:¥3592.00

会员价格:¥3592.00

  • 到货时间:3~4周

    金山科研平台,产品价格货期咨询微信:jinshanbio Source:E. coli-derived, Val2-Val708, with an N-terminal Met and 6-His tag Accession #:YP_001464379 N-terminal Sequence Analysis:Met Purity:>95%, by SDS-PAGE under reducing conditions and visualized by Colloidal Coomassie® Blue stain at 5 µg per lane. Endotoxin Level: Predicted Molecular Mass:82 kDa SDS-PAGE:70-75 kDa, reducing conditions Activity:Measured by its ability to hydrolyze 4-methylumbelliferyl-alpha -D-galactopyranoside.The specific activity is >3,000 pmol/min/µg, as measured under the described conditions. Formulation:Supplied as a 0.2 µm filtered solution in Tris, NaCl and Glycerol.See Certificate of Analysis for details. Molecule Information: alpha-Galactosidase/a-Gal Aliases: rafA Entrez Gene IDs: 5589615 (E. coli) Background: alpha-Galactosidase A 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 1-4)Gal(beta 1-4)Glc (beta 1-1)-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.

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