Recombinant Human beta-Galactosidase/GLB1 (C-6His)
Artikelnummer:
ABI-32-7427-10
Hersteller Artikelnummer:
32-7427-10
Alternativnummer:
ABI-32-7427-10-10UG
Hersteller:
Abeomics
Kategorie:
Proteine/Peptide
Source: Human Cells. MW :74.63kD. 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. beta Galactosidase is a lysosomal beta Galactosidase that hydrolyzes the terminal beta Galactose from Ganglioside and Keratan sulfate. In lysosome, the mature beta Galactosidase protein associates with Cathepsin A and Neuraminidase 1 to form the lysosomal multienzyme complex . An alternative splicing at the RNA level of beta Galactosidase results a catalytically inactive beta Galactosidase that plays an important role in vascular development. Defects of beta-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 beta Galactosidase, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases.
