期刊
CELL BIOLOGY INTERNATIONAL
卷 32, 期 10, 页码 1193-1198出版社
ACADEMIC PRESS LTD ELSEVIER SCIENCE LTD
DOI: 10.1016/j.cellbi.2008.07.021
关键词
Polycystin-2; Centrosome; mitosis; polycystic kidney disease
类别
资金
- poste d'acceuil INSERM
- Deutsche Forschungsgemeinschaft (DFG)
- University of Muenster
- GIS Maladies Rares
- GRIP program from Genzyme
- Association pour l'Information et la Recherche sur les maladies Renales Genetiques (AIRG-France)
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. (c) 2008 International Federation for Cell Biology. Published by Elsevier Ltd. All rights reserved.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据