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首页> 外文期刊>Cell biology international. >Nuclear deformation characterizes Werner syndrome cells.
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Nuclear deformation characterizes Werner syndrome cells.

机译：核变形是Werner综合征细胞的特征。

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摘要

Mutations in the lamin A gene have been shown, among other defects, to give rise to Hutchinson-Gilford progeria syndrome (HGPS) and to atypical Werner syndrome (WS), both of which are progeroid disorders. Here, we have investigated well-characterized WS patient cell strains that are compound heterozygous for mutations in the WRN gene. As in HGPS and in atypical WS, we found nuclear deformations to be characteristic of all cell strains studied. In WS cells centrosome number, assembly of the nuclear lamina and nuclear pore distribution occurred normally. Furthermore, nuclear deformations were not associated with a defect in lamin A expression. We propose that nuclear deformation is a universal characteristic of progeroid cells and may result from slow cell cycle progression.
机译：已经证明，lamin A基因中的突变会导致Hutchinson-Gilford早衰综合症（HGPS）和非典型Werner综合症（WS），这两个都是早衰症。在这里，我们研究了特征明确的WS患者细胞株，它们是WRN基因突变的复合杂合子。像在HGPS和非典型WS中一样，我们发现核变形是所有研究细胞株的特征。在WS细胞中心体数目中，核层的组装和核孔分布正常发生。此外，核变形与lamin A表达的缺陷无关。我们提出核变形是类胚细胞的普遍特征，可能是由于细胞周期进程缓慢所致。

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