Centrosome overduplication and mitotic instability in PKD2 transgenic lines

被引:41
作者
Burtey, Stephane [1 ]
Riera, Marta [1 ]
Ribe, Emilie [1 ]
Pennenkamp, Petra [2 ]
Rance, Roselyne [1 ]
Luciani, Judith [1 ]
Dworniczak, Bernd [2 ]
Mattei, Marie Genevieve [1 ]
Fontes, Michel [1 ]
机构
[1] INSERM, Fac Med Timone, UMR 491, F-13258 Marseille, France
[2] Univ Hosp Muenster, Inst Human Genet, D-48149 Munster, Germany
关键词
Polycystin-2; Centrosome; mitosis; polycystic kidney disease;
D O I
10.1016/j.cellbi.2008.07.021
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
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. (c) 2008 International Federation for Cell Biology. Published by Elsevier Ltd. All rights reserved.
引用
收藏
页码:1193 / 1198
页数:6
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