Centrosome overduplication and mitotic instability in PKD2 transgenic lines.
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.
Burtey
Stéphane
S
INSERM UMR 491, Medical Genetics and Development, Faculté de Médecine de la Timone, Marseille, France.
Riera
Marta
M
Ribe
Emilie
E
Pennenkamp
Petra
P
Rance
Roselyne
R
Luciani
Judith
J
Dworniczak
Bernd
B
Mattei
Marie Geneviève
MG
Fontés
Michel
M
D000782:Aneuploidy; D000818:Animals; D002478:Cells, Cultured; D018385:Centrosome / Q000378:metabolism*; D043171:Chromosomal Instability; D005347:Fibroblasts / Q000166:cytology / Q000502:physiology; D006801:Humans; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D008822:Mice, Transgenic; D008938:Mitosis / Q000235:genetics*; D016891:Polycystic Kidney, Autosomal Dominant / Q000235:genetics / Q000378:metabolism; D008941:Spindle Apparatus / Q000378:metabolism; D050396:TRPP Cation Channels / Q000235:genetics* / Q000378:metabolism; D019076:Transgenes
D050396:TRPP Cation Channels