Centrosome overduplication and mitotic instability in PKD2 transgenic lines
Centrosome overduplication and mitotic instability in PKD2 transgenic lines
复制标题
DOI：
10.1016/j.cellbi.2008.07.021
复制
发表时间：
2008-10-01
影响因子：
3.9
通讯作者：
Fontes, Michel
中科院分区：
文献类型：
--
作者：
Burtey, Stephane;Riera, Marta;Fontes, Michel
polycytin -2 （PC-2）是一种由PKD2编码并参与常染色体显性多囊肾病（ADPKD）的蛋白，是一种非选择性阳离子通道，最近与原发性纤毛的功能有关。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的人类PKD2。两个过表达人类PKD2的转基因小鼠系表现出有丝分裂不稳定性。来自这些转基因小鼠系的成纤维细胞有异常的染色体数目。这些细胞系也有多余的中心体。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发挥了作用，这一假设正在其他模型中进行评估。(c) 2008年国际细胞生物学联合会。Elsevier Ltd.出版。版权所有。
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. (c) 2008 International Federation for Cell Biology. Published by Elsevier Ltd. All rights reserved.