无β脂蛋白血症中红细胞膜脂质的改变：磷脂和脂肪酸组成

Alterations in erythrocyte membrane lipids in abetalipoproteinemia: phospholipid and fatty acyl composition.

作者信息

Iida H, Takashima Y, Maeda S, Sekiya T, Kawade M, Kawamura M, Okano Y, Nozawa Y

出版信息

Biochem Med. 1984 Aug;32(1):79-87. doi: 10.1016/0006-2944(84)90010-3.

Abstract

Scanning electron microscopic observation revealed that there were wide variations including typical acanthocytes in morphology of erythrocytes from a patient with abetalipoproteinemia. The erythrocyte membrane phospholipids and cholesterol contents from a patient was higher by 25% compared to an age-matched control subject. Analysis of phospholipid composition of red blood cells showed an increase of sphingomyelin (25.1----30.1%) with a concomitant decrease of lecithin (27.5----21.0%). Thus, the sphingomyelin/lecithin ratio was increased dramatically (0.91----1.43). As for fatty acyl chain composition of main phospholipids, an increased percentage of palmitic acid and docosahexaenoic acid and a decreased proportion of arachidonic acid and lignoceric acid were observed for sphingomyelin. There was an increment of palmitic acid which was accompanied with a decrease of linoleic acid in lecithin. On the other hand, no significant difference was shown in the fatty acid composition of phosphatidylethanolamine and phosphatidylserine plus phosphatidylinositol between a patient and control.

摘要

扫描电子显微镜观察显示，无β脂蛋白血症患者红细胞的形态存在广泛差异，包括典型的棘红细胞。与年龄匹配的对照受试者相比，患者的红细胞膜磷脂和胆固醇含量高出25%。对红细胞磷脂组成的分析表明，鞘磷脂增加（25.1----30.1%），同时卵磷脂减少（27.5----21.0%）。因此，鞘磷脂/卵磷脂比值显著增加（0.91----1.43）。至于主要磷脂的脂肪酰链组成，鞘磷脂中棕榈酸和二十二碳六烯酸的百分比增加，花生四烯酸和木蜡酸的比例降低。卵磷脂中棕榈酸增加，同时亚油酸减少。另一方面，患者与对照之间的磷脂酰乙醇胺以及磷脂酰丝氨酸加磷脂酰肌醇的脂肪酸组成没有显著差异。