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Centrosome overduplication and mitotic instability in PKD2 transgenic lines.

机译:PKD2转基因品系中的中心体过度复制和有丝分裂不稳定。

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摘要

Polycystin-2 (PC-2), a protein encoded by PKD2 and involved in autosomal dominant polycystic kidney disease (ADPKD), is a non-selective cationic channel recently implicated in the function of primary cilia. We recently constructed a new animal model in the form of a transgenic mouse with a BAC-containing human PKD2 inserted in its genome. Two transgenic mouse lines overexpressing human PKD2 showed mitotic instability. Fibroblasts from these transgenic mouse lines have abnormal chromosomal numbers. These lines also have supernumerary centrosomes. PC-2 overexpression is associated with mitotic instability and centrosome overduplication. PC-2 therefore seems to play a role in centrosome duplication, and this hypothesis is being evaluated in other models.
机译:Polycystin-2(PC-2)是由PKD2编码并参与常染色体显性多囊肾疾病(ADPKD)的蛋白质,是最近与初级纤毛功能相关的非选择性阳离子通道。我们最近以转基因小鼠的形式构建了一种新的动物模型,在其基因组中插入了含有BAC的人PKD2。两种过表达人PKD2的转基因小鼠品系显示有丝分裂不稳定。这些转基因小鼠品系的成纤维细胞具有异常的染色体数。这些系也具有多余的中心体。 PC-2过表达与有丝分裂不稳定和中心体过度复制有关。因此,PC-2似乎在中心体复制中起作用,这一假说正在其他模型中进行评估。

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