83-1174-85　Recombinant Human beta-Galactosidase/GLB1（C-His） 500ug　BP147-500ug

特徴

• 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.

仕様

• サイズ：500ug
• 種由来：Human
• 発現宿主：Human 293 Cells
• エンドトキシン：Less than 0.1 EU/ug
• タグ：C-6His
• Target Sequence：Leu24-Val677
• FormulationLyophilized from a 0.2 um filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.0
• Accession 番号：P16278
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