Figure 1.

SMNΔ7 mouse model exhibits the most severe SMA motor phenotype

(A) Survival (i), body weight (ii) and righting time (iii) of SMNΔ7, Taiwanese, Smn^2B/- mutants with control littermates. Statistics: Mantel-Cox test for survival (i), multiple t-test with Holm-Sidak method for body weight (ii) and righting time (iii); animal numbers (n) = SMNΔ7: control = 25, SMA = 14; Taiwanese: control = 19, SMA = 18; Smn^2B/-: control = 27, SMA = 27.

(B) Western blot analysis of SMN (38 kDa) and tubulin (50 kDa, loading control) protein levels of P10 spinal cords from SMNΔ7, Taiwanese, Smn^2B/- mutants.

(C) Normalized quantification (SMN/tubulin) of Western blot intensities from the same groups as in (B). Statistics: one-way ANOVA with Tukey’s correction. n values = 3 per genotype. Data are presented as mean ± SEM. Asterisks on top of bars without horizontal line indicate significance compared with the control group.

∗p < 0.05; ∗∗p < 0.01; ∗∗∗p < 0.001.