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GLYCOSPHINGOLIPIDS IN FETAL TAY‐SACHS DISEASE BRAIN AND LUNG CULTURES
Author(s) -
Hoffman Linda M.,
Amsterdam D.,
Brooks S. E.,
Schneck L.
Publication year - 1977
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1977.tb10705.x
Subject(s) - tay sachs disease , fetus , ganglioside , lactosylceramide , glycolipid , cerebellum , cell culture , chemistry , biology , biochemistry , medicine , endocrinology , pregnancy , disease , genetics
— A study was undertaken of the glycosphingolipids in cell cultures derived from cerebellum of Tay‐Sachs disease fetal brain in order to determine the suitability of such cell strains as a model for Tay‐Sachs disease. The glycosphingolipids in the Tay‐Sachs disease cultured cerebellar cells were compared with those found in normal cultured cerebellar cells, normal and Tay‐Sachs cultured lung cells, and normal and Tay‐Sachs fetal brain. The glycolipids were separated by TLC, then analyzed by GLC of the trimethylsilyi derivatives of the methylglycosides of the sugar moieties. In the cultured cerebellar lines, the predominant gangliosides were G M2 , G M3 , and G D3 . There was a 4‐fold increase of G M2 in the Tay‐Sachs as compared with the normal line. Only G M3 and G D3 gangliosides were found in the Tay‐Sachs and the normal fetal lung cell cultures. The major neutral glycosphingolipids in all of the cultured cells which were analyzed were glucosylceramide, lactosylceramide, digalactosyl‐glucosylceramide, and globoside. When the Tay‐Sachs cerebellar cells were labelled with [1‐ 14 C]gluco‐samine, some radioactivity was observed in the trihexosylceramide band, indicating the presence of a small amount of a galactosamine‐containing trihexosylceramide which may be asialo‐G M2 (G A2 ). The trihexosylceramide in Tay‐Sachs fetal brain was identified as G A2 by GLC. Both Tay‐Sachs and normal fetal brain gangliosides were more complex than those found in the cultured cells. Long chain fatty acids (C 24:0 and C 24;1 ) predominated in all of the glycosphingolipids of the Tay‐Sachs and the normal cultured cerebellar cells. In contrast, the glycosphingolipids of Tay‐Sachs and normal fetal brain contained mainly the shorter chain fatty acids (C 16 : 0 , C 18 : 0 , and C 18:1 ). The cerebrosides in both the Tay‐Sachs and normal fetal brains were mainly glucosylceramide with only small amounts of the galactosylceramide which predominates in infant brain. Cultured cells from the fetal Tay‐Sachs disease