Fig. 1
Analysis of lifespan, disease onset, and progression in SQSTM1;SOD1H46R mice. a Growth curve in female mice with four different genotypes [wild-type (WT; n = 8–12), SQSTM1 (SQSTM1; n = 9–12), SOD1H46R (H46R; n = 2–12), and SQSTM1;SOD1H46R (SQSTM1;H46R; n = 1–12)]. b Growth curve in male mice with four different genotypes [WT; n = 4–12, SQSTM1; n = 11–12, H46R; n = 2–12, and SQSTM1;H46R; n = 4–12]. a, b Body weight of SQSTM1;SOD1H46R mice was significant lower than that of SOD1H46R mice (*p < 0.05, **p < 0.01, ***p < 0.001, ****p < 0.0001). There are no significantly differences in body weight between WT and SQSTM1 mice. Values were mean ± s.d.. Statistical significance was evaluated by one-way ANOVA with Bonferroni’s post hoc test. c Lifespan analysis of SOD1H46R mice (H46R; n = 31) and SQSTM1;SOD1H46R mice (SQSTM1;H46R; n = 28). Kaplan-Meier analysis demonstrated that lifespan in SQSTM1;SOD1H46R mice (mean ± s.d.; 179.3 ± 2.6 days) was significantly shorter than that in SOD1H46R mice (189.3 ± 2.2 days) (Log-rank test; p = 0.0013). d Onset of the disease in SOD1H46R (H46R; n = 24) and SQSTM1;SOD1H46R (SQSTM1;H46R; n = 24) mice. Onset of disease is defined as the turning-point of body weight successive reduction. Onset in SQSTM1;SOD1H46R mice (mean ± s.d.; 21.5 ± 2.1 weeks) was significantly earlier than that in SOD1H46R mice (23.8 ± 2.1 weeks) (Log-rank test; p = 0.0007). e Survival after the onset of disease (post-onset survival interval) in SOD1H46R (H46R; n = 24) and SQSTM1;SOD1H46R (SQSTM1;H46R; n = 24) mice. Survival after the onset is defined as the period between the onset of disease and end-point. Post-onset survival interval in SQSTM1;SOD1H46R mice (mean ± s.d.; 3.9 ± 1.3 weeks) was significantly longer than that in SOD1H46R mice (3.0 ± 1.1 weeks) (Log-rank test; p = 0.0314)