Centrosome overduplication and mitotic instability in PKD2 transgenic lines.

Abstract:

: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.

journal_name

Cell Biol Int

authors

Burtey S,Riera M,Ribe E,Pennenkamp P,Rance R,Luciani J,Dworniczak B,Mattei MG,Fontés M

doi

10.1016/j.cellbi.2008.07.021

subject

Has Abstract

pub_date

2008-10-01 00:00:00

pages

1193-8

issue

10

eissn

1065-6995

issn

1095-8355

pii

S1065-6995(08)00511-8

journal_volume

32

pub_type

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