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β Galactosidase is a lysosomal β Galactosidase that hydrolyzes the terminal β Galactose from Ganglioside and Keratan sulfate. In lysosome, the mature β Galactosidase protein associates with Cathepsin A and Neuraminidase 1 to form the lysosomal multienzyme complex . An alternative splicing at the RNA level of β Galactosidase results a catalytically inactive β Galactosidase that plays an important role in vascular development. Defects of β-galactosidase (GLB1) are the cause of diseases like GM1-gangliosidosis which is a lysosomal storage disease and Morquio Syndrome B that cause patients to have abnormal elastic fibers. More than 100 mutations have been identified for β Galactosidase, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases.
规格 | 价格 | 库存 | 数量 |
---|---|---|---|
10 μg | ¥ 910 | 5日内发货 | |
50 μg | ¥ 2,760 | 5日内发货 | |
500 μg | ¥ 12,100 | 5日内发货 | |
1 mg | ¥ 17,400 | 5日内发货 |
生物活性 | Activity has not been tested. It is theoretically active, but we cannot guarantee it. If you require protein activity, we recommend choosing the eukaryotic expression version first. |
产品描述 | β Galactosidase is a lysosomal β Galactosidase that hydrolyzes the terminal β Galactose from Ganglioside and Keratan sulfate. In lysosome, the mature β Galactosidase protein associates with Cathepsin A and Neuraminidase 1 to form the lysosomal multienzyme complex . An alternative splicing at the RNA level of β Galactosidase results a catalytically inactive β Galactosidase that plays an important role in vascular development. Defects of β-galactosidase (GLB1) are the cause of diseases like GM1-gangliosidosis which is a lysosomal storage disease and Morquio Syndrome B that cause patients to have abnormal elastic fibers. More than 100 mutations have been identified for β Galactosidase, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases. |
种属 | Human |
表达系统 | HEK293 Cells |
标签 | C-6xHis |
蛋白编号 | P16278 |
别名 | β-Galactosidase,Lactase,GLB1,ELNR1,Elastin Receptor 1,Beta-Galactosidase,Acid β-Galactosidase,Acid Beta-Galactosidase |
氨基酸序列 | Leu24-Val677 |
蛋白构建 | Leu24-Val677 |
蛋白纯度 | Greater than 95% as determined by reducing SDS-PAGE. (QC verified) |
分子量 | 90 KDa (reducing condition) |
内毒素 | < 0.1 ng/µg (1 EU/µg) as determined by LAL test. |
缓冲液 | Supplied as a 0.2 μm filtered solution of 20 mM Tris-HCl, 150 mM NaCl, pH 8.0. |
存储 | Lyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at -80°C. For reconstituted protein solutions, the solution can be stored at -20°C to -80°C for at least 3 months. Please avoid multiple freeze-thaw cycles and store products in aliquots. |
运输方式 | In general, Lyophilized powders are shipping with blue ice. Solutions are shipping with dry ice. |
研究背景 | β Galactosidase is a lysosomal β Galactosidase that hydrolyzes the terminal β Galactose from Ganglioside and Keratan sulfate. In lysosome, the mature β Galactosidase protein associates with Cathepsin A and Neuraminidase 1 to form the lysosomal multienzyme complex . An alternative splicing at the RNA level of β Galactosidase results a catalytically inactive β Galactosidase that plays an important role in vascular development. Defects of β-galactosidase (GLB1) are the cause of diseases like GM1-gangliosidosis which is a lysosomal storage disease and Morquio Syndrome B that cause patients to have abnormal elastic fibers. More than 100 mutations have been identified for β Galactosidase, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases. |
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