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• W2049789958 abstract "The concentration of N-acetylneuraminic acid (NANA) in the non-dialyzable and dialyzable glycopeptides derived from Tay-Sachs brain tissue was the same as that found in normal controls. This suggests that the heteropolysaccharide chains of the brain glycoproteins do not contain the structural feature which is responsible for the accumulation of Tay-Sachs ganglioside. It is known that Tay-Sachs ganglioside becomes elevated in this disease due to the terminal location of N-acetylgalactosamine which cannot be cleaved due to the deficiency of hexosaminase A. Gangliosidic NANA becomes elevated since the cleavage of this sugar is dependent upon the prior removal of the terminal N-acetylgalactosamine. The concentration and carbohydrate composition of the non-dialyzable glycopeptides from cerebral gray matter in Tay-Sachs disease do not differ from that found in controls, although there was a 4-fold increase in gangliosidic NANA in the same tissu sample. The concentration of hexosamine and hexose in the dialyzable glycopeptides which do not contain NANA is elevated in Tay-Sachs disease. This suggests that hexosaminidase A, absent in Tay-Sachs disease, participate in the catabolic breakdown of the heteropolysaccharide chains of the brain glycoproteins. The concentration of the glycosaminoglycans is not affected in Tay-Sachs disease." @default.
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• W2049789958 date "1972-03-01" @default.
• W2049789958 modified "2023-10-17" @default.
• W2049789958 title "Glycoproteins in Tay-Sachs disease: Isolation and carbohydrate composition of glycopeptides" @default.
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• W2049789958 doi "https://doi.org/10.1016/0006-8993(72)90596-3" @default.
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