beta-Galactosidase-1/GLB1: Proteins and Enzymes

beta-Galactosidase-1 (GLB1) is a lysosomal beta-galactosidase that hydrolyzes the terminal beta-galactose from ganglioside and keratan sulfate. Defects in this gene are the causes of lysosomal storage diseases for GM1-gangliosidosis and Morquio B syndrome (also known as mucopolysaccharidosis IVB). In GM1 gangliosidosis, GM1 ganglioside accumulates in the neurons of the central nervous system, because of the deficiency (0 plus or minus 3% of normal) of lysosomal beta-galactosidase activity. GM1 ...