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. Author manuscript; available in PMC: 2026 Jul 8.
Published in final edited form as: Cell. 2026 Mar 11;189(6):1656–1679.e42. doi: 10.1016/j.cell.2026.02.008

Figure 5. Sildenafil extends the lifespan of small and large mammalian models of LS.

Figure 5.

(A) Kaplan–Meier survival curve of untreated Ndufs4 KO mice (10 females, 8 males) compared to Ndufs4 KO mice exposed to sildenafil citrate added in the drinking water (6 females, 6 males). ***p<0.001; Log-rank (Mantel-Cox) test. (B-C) Functional parameters in untreated or sildenafil-treated 42-45-days-old Ndufs4 KO mice (n=15 mice out of five independent experiments) collected by placing mice in a metabolic chamber. **p<0.01, ***p<0.001, ns (not significant); ANOVA posthoc analysis. (D-E) Representative immunohistochemistry and related quantification of positive for cleaved caspase3, Iba1, and Prkg1 in Purkinje cells in untreated or sildenafil-treated 50-days-old Ndufs4 KO mice (n=5-6). **p<0.01, ***p<0.001, ns; ANOVA post-hoc analysis. (F) Kaplan–Meier survival curve of wild-type (WT) piglets (n=10), untreated SURF1 KO piglets (n=16), and SURF1 KO piglets treated with sildenafil (n=3 with 2.1 mg/kg/day, n=4 with 0.5 mg/kg/day). *p<0.05 treated mutants vs. untreated; Log-rank (Mantel-Cox) test. (G) Quantification of body temperature in SURF1 KO piglets. ****p<0.0001, ns; unpaired two-tailed t-test. (H) Neurological score of SURF1 KO piglets. ****p<0.0001, *p<0.05, ns; unpaired two-tailed t-test. (I-J) Representative immunoblot image and related quantification of PRKG1 protein expression in basal ganglia lysates of SURF1 KO piglets. n=3 replicates per sample in two independent experiments. **p<0.01, *p<0.05, ns; unpaired two-tailed t-test.