Abstract：
:Cultured cells derived from Tay-Sachs disease (TSD) fetal cerebellum were shown to accumulate GM2 ganglioside when compared with control cultures. In contrast, fibroblasts derived from TSD fetal lung do not contain GM2. GM2 was identified by thin-layer chromatography (TLC) and confirmed by gas-liquid chromatography (GLC). Unlike fetal TSD brain, the cell cultures established from fetal TSD brain have high concentrations of globoside and GD3 and little or no asialo GM2(GA2). The GM2 and related glycosphingolipids in these cultured cells contain a high percentage of C24:0 and C24:1 fatty acids. In spite of these differences, this TSD cell strain is unique in that it accumulates GM2, and can therefore serve as a useful in vitro model for the study of TSD.
journal_name
Brain Resjournal_title
Brain researchauthors
Hoffman LM,Amsterdam D,Schneck Ldoi
10.1016/0006-8993(76)91052-0subject
Has Abstractpub_date
1976-07-23 00:00:00pages
109-17issue
1eissn
0006-8993issn
1872-6240pii
0006-8993(76)91052-0journal_volume
111pub_type
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