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

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Science  28 Feb 1969:
Vol. 163, Issue 3870, pp. 946-947
DOI: 10.1126/science.163.3870.946


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.

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