产品描述 概述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. 使用说明This material is offered by Novin Biotech for research, laboratory or further evaluation purposes. NOT FOR HUMAN USE. 技术规格Tag | C-6His | 种属 | Human | 表达系统 | Human cells | Accession# | P16278 | Source | Human Cells | Formulation_Description | Supplied as a 0.2 μm filtered solution of 20mM TrisHCl, 150mM NaCl, pH 8.0. | Storage | Store at < -20°C, stable for 6 months after receipt.Please minimize freeze-thaw cycles. | Purity | Greater than 95% as determined by reducing SDS-PAGE. | Endotoxin | Less than 0.1 ng/μg (1 EU/μg) as determined by LAL test. | 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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