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Science 28 February 1969:
Vol. 163. no. 3870, pp. 946 - 947
DOI: 10.1126/science.163.3870.946
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Articles

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

Monica C. MacBrinn 1, Shintaro Okada 1, Mae Wan Ho 1, Chin Chai Hu 1, and John S. O'Brien 1

1 Department of Neurosciences, University of California at San Diego, La Jolla 92037

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


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
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Ultrastructural Ocular Pathology of Hunter's Syndrome: Systemic Mucopolysaccharidosis Type II.
T. M. Topping, K. R. Kenyon, M. F. Goldberg, and A. E. Maumenee (1971)
Arch Ophthalmol 86, 164-177
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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
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GM1-Gangliosidosis: Correlation of Clinical and Biochemical Data.
Y. Suzuki, A. C. Crocker, and K. Suzuki (1971)
Arch Neurol 24, 58-64
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Science. ISSN 0036-8075 (print), 1095-9203 (online)