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. 2015 Oct;185(10):2843–2860. doi: 10.1016/j.ajpath.2015.06.014

Figure 7.

Figure 7

Allelic increase in the PKD2 transgene remarkably improves the prognosis of Pkd2-deficient mice. A: Kaplan-Meier survival analysis of wild-type (WT), Pkd2−/−;PKD2tg, and Pkd2−/−;PKD2tg/tg mice. Pkd2-null mice with homozygous PKD2 transgenes (Pkd2−/−;PKD2tg/tg) show a much higher survival rate than Pkd2-null mice with the hemizygous PKD2 transgene (Pkd2−/−;PKD2tg). Median survival rate of the Pkd2−/−;PKD2tg/tg mice is approximately 12 months, whereas that of Pkd2−/−;PKD2tg mice is around 6 months. B: Kidney/body ratio (kidney index) of 1-, 3-, 6-, 9-, and 12-month-old wild-type, Pkd2−/−;PKD2tg, and Pkd2−/−;PKD2tg/tg mice. C: Liver/body ratio (liver index) of the same mice. D–AA: Cystic phenotypes in the Pkd2−/−;PKD2tg/tg kidney, liver, and pancreas. No obvious gross cysts are found in the Pkd2−/−;PKD2tg/tg kidneys up to the age of 12 months (D–G). Histological analysis reveals similar findings (H–K), but sporadic histological cysts are found in the kidney of 12-month-old Pkd2−/−;PKD2tg/tg mice (K). No gross cysts are seen in the 3- and 6-month-old liver (L and M), but they are seen in the 9- and 12-month-old liver (arrows) (N and O). Histological examination reveals cystic lesions in the Pkd2−/−;PKD2tg/tg liver at 6 months (Q), and the cystic phenotype worsens with age (R and S). There are no obvious gross or histological cysts in the pancreas of these mice (T–AA). High-power images of the boxed areas are shown in the lower-left corners. BB: Blood urea nitrogen (BUN) levels of 1-, 3-, 6-, 9-, and 12-month-old wild-type, Pkd2−/−;PKD2tg, and Pkd2−/−;PKD2tg/tg mice. Significantly higher elevations are detected in the 9- and 12-month-old Pkd2−/−;PKD2tg mice compared to age-matched wild-type and Pkd2−/−;PKD2tg/tg mice, respectively. There is no significant difference between the BUN levels in wild-type and Pkd2−/−;PKD2tg/tg mice. CC: Similar findings are obtained in the creatinine (CRE) analysis of the same mice. However, a significant difference between the CRE levels of wild-type and Pkd2−/−;PKD2tg/tg mice is found only at the age of 12 months. ∗P < 0.05, ∗∗P < 0.01, and ∗∗∗P < 0.001. n = 3 (B, wild-type); n = 5 (B, Pkd2−/−;PKD2tg and Pkd2−/−;PKD2tg/tg; D–AA, each age); n = 25 (A, wild-type and Pkd2−/−;PKD2tg); n = 40 (A, Pkd2−/−;PKD2tg/tg). Scale bars: 2 mm (D–G); 4 mm (L–O); 1 mm (T–W); 500 μm (H–K, P–S, X–AA); 100 μm (insets). M, months.