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.
パッケージサイズ | 在庫状況 | 単価(税別) |
---|---|---|
10 μg | 約5 days | ¥ 33,000 |
50 μg | 約5 days | ¥ 100,000 |
500 μg | 約5 days | ¥ 436,000 |
1 mg | 約5 days | ¥ 626,500 |
説明 | 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. |
Species | Human |
Expression Host | HEK293 Cells |
Tag | C-6xHis |
Accession Number | P16278 |
別名 | Lactase, Acid Beta-Galactosidase, Elastin Receptor 1, GLB1, Acid β-Galactosidase, β-Galactosidase, ELNR1, Beta-Galactosidase |
Amino Acid | Leu24-Val677 |
Construction | Leu24-Val677 |
Protein Purity | Greater than 95% as determined by reducing SDS-PAGE. (QC verified) |
分子量 | 90 KDa (reducing condition) |
Endotoxin | < 0.1 ng/µg (1 EU/µg) as determined by LAL test. |
Formulation | Supplied as a 0.2 μm filtered solution of 20 mM Tris-HCl, 150 mM NaCl, pH 8.0. |
Stability & Storage |
Lyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at-80℃. For reconstituted proteinsolutions, 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. |
Shipping |
In general, Lyophilized powders are shipping with blue ice. Solutions are shipping with dry ice. |
Research Background | 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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Please read the User Guide of Recombinant Proteins for more specific information.
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