Weitzdoerfer Rachel, Fountoulakis Michael, Lubec Gert
Department of Neonatology, University of Vienna, A-1090 Vienna, Austria.
Biochem Biophys Res Commun. 2002 May 3;293(2):836-41. doi: 10.1016/S0006-291X(02)00291-7.
Down syndrome (DS) patients present with morphological abnormalities in brain development, leading to mental retardation. Given the importance of actin cytoskeleton to form the basis of various cell functions, the regulation of actin system is crucial during brain development. We therefore aimed to study the expression levels of actin binding proteins in fetal DS and control cortex. We evaluated the levels of eight actin binding proteins using the proteomic approach of two-dimensional gel electrophoresis with subsequent mass spectroscopical identification of protein spots. In fetal DS brain we found a significant reduction of the actin-related protein complex 2/3 (Arp2/3) 20 kDa subunit and the coronin-like protein p57, which are involved in actin filament cross-linking and nucleation and capping of actin filaments. We conclude that deficient levels of these proteins may, at least partially, be involved in the dysgenesis of the brain in DS.
唐氏综合征(DS)患者存在大脑发育的形态学异常,导致智力迟钝。鉴于肌动蛋白细胞骨架对形成各种细胞功能基础的重要性,肌动蛋白系统的调节在大脑发育过程中至关重要。因此,我们旨在研究胎儿DS和对照皮质中肌动蛋白结合蛋白的表达水平。我们使用二维凝胶电泳的蛋白质组学方法,随后对蛋白质斑点进行质谱鉴定,评估了八种肌动蛋白结合蛋白的水平。在胎儿DS大脑中,我们发现肌动蛋白相关蛋白复合体2/3(Arp2/3)20 kDa亚基和类冠蛋白p57显著减少,它们参与肌动蛋白丝的交联、成核以及肌动蛋白丝的封端。我们得出结论,这些蛋白质水平的缺乏可能至少部分地参与了DS患者大脑的发育异常。