Recombinant Human B-Gal/GLB1 Protein, His Tag

GLB1

P16278

< 1.0 EU per ug of the protein as determined by the LAL method.

Protein A/G

Galactosidase is a lysosomal Galactosidase that hydrolyzes the terminal Galactose from Ganglioside and Keratan sulfate. In lysosome, the mature Galactosidase protein associates with Cathepsin A and Neuraminidase 1 to form the lysosomal multienzyme complex . An alternative splicing at the RNA level of Galactosidase results a catalytically inactive Galactosidase that plays an important role in vascular development. Defects of 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 1 mutations have been identified for 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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