文章基本信息

标题：Abnormal fatty acid composition of erythrocyte glycosphingolipids in congenital dyserythropoietic anemia type II.
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作者：J F Bouhours ; D Bouhours ; J Delaunay 等
期刊名称：JLR Papers In Press
印刷版ISSN：0022-2275
电子版ISSN：1539-7262
出版年度：1985
卷号：26
期号：4
页码：435-441
语种：English
出版社：American Society for Biochemistry and Molecular Biology
摘要：Glycosphingolipids were isolated from the erythrocytes of three siblings clinically affected with congenital dyserythropoietic anemia type II (CDA-II) as well as from the erythrocytes of their parents and of normal individuals. Glycolipids were analyzed by thin-layer chromatography of their native and O-acetylated form and by high performance liquid chromatography of their O-acetyl-N-p-nitrobenzoyl derivatives. The glycolipid content of the CDA-II erythrocytes was 2.5 to 3 times higher than normal. This abnormal concentration resulted from a moderate accumulation of all the glycolipids present in normal erythrocytes and from a strong increase (at least 10 times over normal level) of lactotriaosyl- and lactoneotetraosylceramide. Glycolipids contained higher amounts of long chain fatty acids (C22-C24) in CDA-II patients than in controls. Free ceramide content of CDA-II erythrocytes was two times greater than the control value and the fatty acid composition was also altered.