Figure 6. Expression of XBP1s suppresses cyst formation in Prkcsh mutant mice.
(A) PC1 processing in Prkcsh-deficient cells stably expressing Pkd1F/H-BAC is improved by expression of XBP1s. XBP1s overexpression was achieved by recombinant adenovirus infection. (B) XBP1s expression in the kidney of ROSA26-XBP1s lox-stop-lox transgenic mice with or without Ksp-Cre transgene analyzed at P42 by Western blotting showing XBP1s expression in response to CRE activity. (C) XBP1s (indicated by arrowheads) was detected by immunofluorescence in the DBA-positive collecting duct segments where the Ksp-Cre is expressed. (D) Representative images of kidney sections with the indicated genotype showing improvement of polycystic kidney disease with expression of XBP1s. (E) Aggregate data for kidney weight/body weight ratio, cystic index, and BUN levels in the mice of genotypes indicated in the key in D. n (from left to right) = 3, 3, 6; results are shown as mean ± SEM (ANOVA); ***P < 0.001; **P < 0.01.