Cerebral GM1-Gangliosidosis: Chemical Pathology of Visceral Organs

Science  29 Mar 1968:
Vol. 159, Issue 3822, pp. 1471-1472
DOI: 10.1126/science.159.3822.1471


The livers and spleens from three patients with cerebral GM1-gangliosidosis contained greatly increased concentrations of a mucopolysaccharide tentatively identified as keratan sulfate. The concentration of a very soluble sialomucopolysaccharide was also increased. Concentrations of these compounds were not increased in the viscera of patients with Tay-Sachs disease (GM2-gangliosidosis). GM1-gangliosidosis appears to be a combined cerebral gangliosidosis and visceral mucopolysaccharidosis.