Fig. 4: Expression of CTT partially suppresses cystic disease in a rapidly progressive orthologous mouse model of ADPKD when generated on an NNT-competent background. | Nature Communications

Fig. 4: Expression of CTT partially suppresses cystic disease in a rapidly progressive orthologous mouse model of ADPKD when generated on an NNT-competent background.

From: The C-terminal tail of polycystin-1 suppresses cystic disease in a mitochondrial enzyme-dependent fashion

Fig. 4: Expression of CTT partially suppresses cystic disease in a rapidly progressive orthologous mouse model of ADPKD when generated on an NNT-competent background.The alternative text for this image may have been generated using AI.

A Development of the 2HA-PC1-CTT;Pkd1fl/fl;Pkhd1-Cre mouse model. We crossed the 2HA-PC1-CTT;Pkd1fl/fl;Pax8rtTA;TetO-Cre mouse model on the “N” background (N-Pkd1-KO + CTT) with the previously characterized developmental Pkd1fl/fl;Pkhd1-Cre (Pkhd1-Cre;Pkd1-KO)22,23,24,41 ADPKD mouse model, generated on the “J” background. Breeders with a Pkd1fl/+;Pkhd1-Cre genotype were used, due to the constitutive activity of the Pkhd1-Cre that initiates collecting duct-specific Cre-mediated recombination during embryonic stages and prevents animals with the complete Pkd1fl/fl;Pkhd1-Cre from reaching sexual maturity. B Comparative analysis of NJ F1 (purple box) generation mice showing differences in KW/BW ratio. Of note, no phenotype differences were observed in NJ WT mice that do or do not express CTT (NJ;Pkhd1-Cre + CTT and NJ;WT, respectively). Red dots represent the animals depicted in (E). C Comparative analysis of NN F2 (blue box) generation mice showing differences in KW/BW ratio. Of note, no phenotype differences were observed in NN WT mice that do or do not express CTT (NN;Pkhd1-Cre + CTT and NN;WT, respectively). Red dots represent the animals depicted in (F). D Comparative analysis of JJ F2 (red box) generation mice showing differences in KW/BW ratio. Of note, no phenotype differences were observed in JJ WT mice that do or do not express CTT (JJ;Pkhd1-Cre + CTT and JJ;WT, respectively). Red dots represent the animals depicted in (G). E–G H&E-stained kidney sections (4×) from p14 NJ F1 (purple box), NN F2 (blue box), and JJ F2 (red box) mice. Each group of sections is representative of the average KW/BW ratio of the entire cohort. Scale bar: 2 mm. Data are depicted with the mean ± SEM. Multiple group comparisons were performed using one-way ANOVA followed by Tukey’s multiple-comparisons test. H&E-stained kidney sections from all of the cystic mice included in these 3 cohorts are provided in Supplementary Fig. 7. Source data are provided as a Source Data file.

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