文章基本信息

标题：Abnormalities in the Membrane of Erythrocytes from Two Patients Homozygous for Familial Hypobetalipoproteinemia
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作者：Akira TAMURA ; Keiji KANDA ; Tatsuzo FUJII 等
期刊名称：Journal of Clinical Biochemistry and Nutrition
印刷版ISSN：0912-0009
电子版ISSN：1880-5086
出版年度：1988
卷号：5
期号：2
页码：103-109
DOI：10.3164/jcbn.5.103
出版社：The Society for Free Radical Research Japan
摘要：Biochemical and morphological investigations were made on erythrocytes from two patients homozygous for genetic hypobetalipoproteinemia. Increased ratios of cholesterol/total phospholipids and sphingomyelin/phosphatidylcholine, and decreased membrane lipid fluidity were detected in the patients' cells as compared with those from a normal adult. Typical acanthocytes were observed which comprised 10-15% of each cell population of the patients. Such acanthocytosis was not corrected by incubation of the cells with either normal plasma, purified LDL, or phosphatidylcholine liposomes, but was corrected by treatment with phospholipase C which caused stomatocytosis of the cells. Incubation of the patients' cells with echinocytogenic agents such as flufenamic acid and lysophosphatidylcholine yielded more intensive echinocytosis than occurred with normal cells. Incubation of the former with a stomatocytogenic reagent, chlorpromazine, yielded weaker stages of stomatocytes than in the case of the latter. These results suggest that the acanthocyte shape may not simply result from the abnormal lipid contents of the cell membrane, and that not only the observed acanthocytes are abnormal but also the other normal-looking cells of the patients may have some abnormalities in their membrane structure.
关键词：hypobetalipoproteinemia;erythrocyte abnormalities;erythrocyte shape;membrane lipids;acanthocyte