Generalized Gangliosidosis: Impaired Cleavage of Galactose from a Mucopolysaccharide and a Glycoprotein

We have demonstrated a tenfold (or greater) reduction of the cleavage of galactose from a mucopolysaccharide and a glycoprotein in generalized gangliosidosis, a lysosomal β-galactosidase deficiency disease. The bony abnormalities in this disease may be due to defective catabolism of mucopolysaccharides in connective tissue.