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胎儿唐氏综合征大脑中埃兹蛋白的多方面减少

Manifold reduction of moesin in fetal Down syndrome brain.

作者信息

Lubec B, Weitzdoerfer R, Fountoulakis M

机构信息

Department of Neonatology, University of Vienna, Vienna, A-1090, Austria.

出版信息

Biochem Biophys Res Commun. 2001 Sep 7;286(5):1191-4. doi: 10.1006/bbrc.2001.5520.

Abstract

Moesin is a member of the ERM family and is involved in plasma membrane-actin cytoskeleton cross-linking, resulting cell adhesion, shape, and motility. Because moesin was shown to be highly expressed in growth cones and moesin/radixin suppression led to impaired structure and function of this key element in brain development, we tested the ERM family, ezrin, radixin, and moesin, in fetal Down syndrome (DS) cortex at the early second trimester. We applied two-dimensional gel electrophoresis with subsequent MALDI detection and identification of protein spots followed by quantification with specific software. Moesin was shown to be significantly and manifold reduced in fetal DS brain, whereas reduction of ezrin and radixin did not reach statistical significance. We therefore propose the involvement of moesin in developmental impairment of DS brain, including deteriorated arborisation, neuritic outgrowth, and neuronal migration. Furthermore, decreased moesin is the second F-actin bundling protein, besides drebrin, that is manifold reduced in fetal DS brain.

摘要

埃兹蛋白是ERM家族的成员,参与质膜-肌动蛋白细胞骨架交联,从而影响细胞黏附、形状和运动。由于埃兹蛋白在生长锥中高表达,且埃兹蛋白/根蛋白抑制会导致脑发育中这一关键元件的结构和功能受损,我们在孕中期早期对胎儿唐氏综合征(DS)皮质中的ERM家族成员埃兹蛋白、根蛋白和埃兹蛋白进行了检测。我们采用二维凝胶电泳,随后进行基质辅助激光解吸电离飞行时间质谱(MALDI)检测和蛋白质斑点鉴定,并用特定软件进行定量分析。结果显示,胎儿DS脑中的埃兹蛋白显著且大幅减少,而埃兹蛋白和根蛋白的减少未达到统计学意义。因此,我们认为埃兹蛋白参与了DS脑的发育障碍,包括树突分支恶化、神经突生长和神经元迁移。此外,除了脑桥蛋白外,埃兹蛋白减少是胎儿DS脑中大幅减少的第二种F-肌动蛋白成束蛋白。

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