Polycystic kidneys caused by sustained expression of Cux1 isoform p75
C Cadieux, R Harada, M Paquet, O Cote… - Journal of Biological …, 2008 - jbc.org
The transcriptional regulator Cux1 (CDP, Cutl1) is aberrantly expressed in mouse models for
polycystic kidney disease. Here we show that p75-Cux1, the shortest isoform of Cux1,
transcribed from an alternative promoter within intron 20, is also deregulated in polycystic
kidneys derived from Pkd1 mutant embryos. To determine the role of the p75-Cux1 isoform
in cystogenesis, we generated transgenic mice expressing p75-CUX1 in the kidneys and
other tissues. Strikingly, these animals developed polycystic kidneys at variable penetrance …
polycystic kidney disease. Here we show that p75-Cux1, the shortest isoform of Cux1,
transcribed from an alternative promoter within intron 20, is also deregulated in polycystic
kidneys derived from Pkd1 mutant embryos. To determine the role of the p75-Cux1 isoform
in cystogenesis, we generated transgenic mice expressing p75-CUX1 in the kidneys and
other tissues. Strikingly, these animals developed polycystic kidneys at variable penetrance …