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. 2016 Nov 23;26(2):354–366. doi: 10.1093/hmg/ddw392

Figure 5.

Figure 5.

Expression of BAC transgene carrying Flcn K508R mutant allele partially, but not completely rescues the kidney-targeted Flcn knockout mouse phenotype. (A) Histology of Flcn-deficient kidney with Flcn K508R mutant expression (Flcn f/d/K508R, CDH16-Cre) at 3 weeks of age compared to Flcn-deficient kidney (Flcn f/d, CDH16-Cre) and control kidney (Flcnf/+, CDH16-Cre). Fewer cysts develop in Flcn f/d/K508R, CDH16-Cre mice compared to Flcn f/d, CDH16-Cre mice with no protruding hyperplastic cells. (B) Kidney to body weight ratio of kidney-targeted Flcn knockout mice with Flcn K508R mutant expression (Flcn f/d/K508R, CDH16-Cre) at 3 weeks of age showed significantly decreased % kidney/body weight relative to kidney-targeted Flcn knockout mice (Flcn f/d, CDH16-Cre). (C) Kaplan-Meier survival plot of kidney-targeted Flcn knockout mice with Flcn K508R mutant expression demonstrates 3-fold increase in survival (61 vs 20 days) compared to kidney-targeted Flcn knockout mice; n= 8 mice for each genotype. (D) Western blot analysis of mTORC1 downstream effectors in Flcn-deficient kidneys with Flcn K508R mutant expression, Flcn-deficient kidneys and control kidneys at 3 weeks of age showing suppression of the mTOR pathway activation in the Flcn f/d/K508R, CDH16-Cre mice compared with Flcn f/d, CDH16-Cre mice. N.S., no significance.