Recombinant Human GLB1/beta-Galactosidase-1

英文名 ：Recombinant Human GLB1/beta-Galactosidase-1

规格 ：10ug/50ug/500ug/1mg

Recombinant Human GLB1/beta-Galactosidase-1  (C473)

 

产品说明(Description)

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. 

Accession #: P16278

Known as: Beta-Galactosidase; Acid Beta-Galactosidase; Lactase; Elastin Receptor 1; GLB1; ELNR1

 

制剂（Formulation）

Supplied as a 0.2 μm filtered solution of 20mM TrisHCl, 150mM NaCl, pH 8.0.

 

质量控制（Quality Control） 

 

Purity:            Greater than 95% as determined by reducing SDS-PAGE.    

Endotoxin:    Less than 0.1 ng/ug (1 EU/ug) as determined by LAL test.

 

 

运输(Shipping)

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

 

保存(Storage)

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.     

 

背景（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.

 

电泳(SDS-PAGE)

 

FOR RESEARCH USE ONLY