Centrosome overduplication and mitotic instability in PKD2 transgenic lines

Autor: Stéphane Burtey, Marta Riera, Marie Geneviève Mattei, Petra Pennenkamp, Roselyne Rance, Michel Fontes, Bernd Dworniczak, Emilie Ribe, Judith Luciani
Rok vydání: 2008
Předmět:
Zdroj: Cell Biology International. 32:1193-1198
ISSN: 1065-6995
DOI: 10.1016/j.cellbi.2008.07.021
Popis: 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.
Databáze: OpenAIRE