Cerebral GM1-gangliosidosis: chemical pathology of visceral organs.
The livers and spleens from three patients with cerebral G(M1)-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 (G(M2)-gangliosidosis). G(M1)-gangliosidosis appears to be a combined cerebral gangliosidosis and visceral mucopolysaccharidosis.
D001927:Brain Diseases; D002855:Chromatography, Thin Layer; D005690:Galactose / Q000032:analysis; D005732:Gangliosides; D006025:Glycosaminoglycans / Q000032:analysis; D006595:Hexosamines / Q000032:analysis; D006801:Humans; D008064:Lipidoses; D008099:Liver / Q000032:analysis*; D011134:Polysaccharides / Q000032:analysis; D013154:Spleen / Q000032:analysis*
D005732:Gangliosides; D006025:Glycosaminoglycans; D006595:Hexosamines; D011134:Polysaccharides; D005690:Galactose