Inserm, Institut national de la santé et de la recherche médicale
Faculté de pharmacie, Aix Marseille Université

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Centrosome overduplication and mitotic instability in PKD2 transgenic lines

Burtey S, Riera M, Ribe E, Pennenkamp P, Rance R, Luciani J, Dworniczak B, Mattei MG, Fontés M. Cell Biol Int. 2008 Oct ;32(10):1193-8. doi : 10.1016/j.cellbi.2008.07.021. Epub 2008 Aug 6.


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.