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GLB1 Protein, Human, Recombinant (His)

GLB1 Protein, Human, Recombinant (His)

产品编号 TMPJ-00869
别名: Lactase, Acid Beta-Galactosidase, Elastin Receptor 1, GLB1, Acid β-Galactosidase, β-Galactosidase, ELNR1, Beta-Galactosidase

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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GLB1 Protein, Human, Recombinant (His)
规格 价格/CNY 货期 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
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产品目录号及名称: GLB1 Protein, Human, Recombinant (His) (TMPJ-00869)
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产品性质
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 Human Cells
标签 C-6His
蛋白编号 P16278
别名 Lactase, Acid Beta-Galactosidase, Elastin Receptor 1, GLB1, Acid β-Galactosidase, β-Galactosidase, ELNR1, Beta-Galactosidase
氨基酸序列 Leu24-Val677
蛋白构建 Recombinant Human Beta-Galactosidase is produced by our Mammalian expression system and the target gene encoding Leu24-Val677 is expressed with a 6His tag at the C-terminus.
蛋白纯度 Greater than 95% as determined by reducing SDS-PAGE. (QC verified)
分子量 90 KDa, reducing conditions
内毒素 Less than 0.1 ng/µg (1 EU/µg) as determined by LAL test.
缓冲液 Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.0.
存储

Store at ≤-70°C, stable for 6 months after receipt. Store at ≤-70°C, stable for 3 months under sterile conditions after opening. Please minimize freeze-thaw cycles.

运输方式

The product is shipped on dry ice/polar packs. Upon receipt, store it immediately at the temperature listed below.

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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Keywords

GLB1 Protein, Human, Recombinant (His) Lactase Acid Beta-Galactosidase Elastin Receptor 1 b-Galactosidase ELNR 1 GLB1 Acid β-Galactosidase GLB 1 β-Galactosidase ELNR1 Acid b-Galactosidase Beta-Galactosidase GLB-1 ELNR-1 recombinant recombinant-proteins proteins protein

 

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