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Science 29 March 1968:
Vol. 159. no. 3822, pp. 1471 - 1472
DOI: 10.1126/science.159.3822.1471
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Articles

Cerebral GM1-Gangliosidosis: Chemical Pathology of Visceral Organs

Kunihiko Suzuki 1

1 Saul R. Korey Department of Neurology, Albert Einstein College of Medicine, Bronx, New York

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.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Paradoxical influence of acid {beta}-galactosidase gene dosage on phenotype of the twitcher mouse (genetic galactosylceramidase deficiency).
J. Tohyama, M. T. Vanier, K. Suzuki, T. Ezoe, J. Matsuda, and K. Suzuki (2000)
Hum. Mol. Genet. 9, 1699-1707
   Abstract »    Full Text »    PDF »
GM1-Gangliosidosis: Ocular and Pathological Manifestations.
J. M. Emery, W. R. Green, R. G. Wyllie, and R. R. Howell (1971)
Arch Ophthalmol 85, 177-187
   Abstract »    PDF »
GM1-Gangliosidosis: Correlation of Clinical and Biochemical Data.
Y. Suzuki, A. C. Crocker, and K. Suzuki (1971)
Arch Neurol 24, 58-64
   Abstract »    PDF »
Tay-Sachs Disease: Demonstration of the Stored Ganglioside in Cultured Cells From Brain Biopsy.
U. Batzdorf, L. L. Sarlieve, V. A. Gold, and J. H. Menkes (1969)
Arch Neurol 20, 650-652
   Abstract »    PDF »
Generalized Gangliosidosis: Impaired Cleavage of Galactose from a Mucopolysaccharide and a Glycoprotein.
M. C. MacBrinn, S. Okada, M. W. Ho, C. C. Hu, and J. S. O'Brien (1969)
Science 163, 946-947
   Abstract »    PDF »


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