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 Intjournal_title
Cell biology internationalauthors
Burtey S,Riera M,Ribe E,Pennenkamp P,Rance R,Luciani J,Dworniczak B,Mattei MG,Fontés Mdoi
10.1016/j.cellbi.2008.07.021subject
Has Abstractpub_date
2008-10-01 00:00:00pages
1193-8issue
10eissn
1065-6995issn
1095-8355pii
S1065-6995(08)00511-8journal_volume
32pub_type
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