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By C.B. Anfinsen (Eds.)

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By C.B. Anfinsen (Eds.)

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The initial steps in the catabolism of gangliosides G and . cells. Since all of the gangliosides whose oligo­ saccharide chain is longer than lactose, are lacking, it was anticipated that something was wrong with the addition of the molecule of N-acetylgalactosamine. The reaction in question is the transfer of N-acetyl­ galactosamine from UDP-N-acetylgalaetosamine to hematoside (Fig. 3 0 ) . The activity of this aminosugar D l a CER - G L U - G A L - N A N A + UDP - GALNAc — • CER-GLU-GAL-GALNAc + UDP NANA Fig.

W . ) ; however, it can be dissociated by various techniques to subunits as small f 56 CURRENT TOPICS IN BIOCHEMISTRY as 32-36S (3 χ 1 0 ^ m . w . ) . Nevertheless, even if the true molecular weight of the genome is 3 χ 1 0 ^ , a m o l ­ ecular weight of 3 χ 1 0 ^ for the product is still far too small. It is therefore not clear whether the en­ tire genome is being copied or whether only portions of the genome are copied. Duesberg (7) did the following experiment, which suggests that the former alternative is the case.

Science, 1 5 5 , 86 (1967). 15. , Sphingolipid 46 CURRENT TOPICS IN BIOCHEMISTRY hydrodases in leukemic leukocytes. 27, 1312 (1967). Cancer R e s . , 16. , Gaynor, E . , Gaucher cells in chronic myelogenous leukemia: an acquired abnormality. Blood, 3 3 , 379 (1969). 17. , Preparation of radioactive TaySachs ganglioside labeled in the sialic acid moiety. J. Lipid R e s . , 1 1 , 144 (1970). 18. , Studies on the metabolism of TaySachs ganglioside. , 2 8 , 596 (1969) . 19. , Demonstration of an alteration of ganglioside metabolism in Tay-Sachs disease.

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