hSOD1G93A mice lacking TLR4 (hSOD1G93A × TLR4−/−) have extended survival and improvements in hind-limb grip strength when compared to hSOD1G93A mice. (A) shows a Kaplan-Meier plot of ages (in days) in which hSOD1G93A mice with normal (TLR4+/+, red line) or fully deleted (TLR4−/−, blue line) TLR4 reached the end-stage of disease (complete hind-limb paralysis and an inability to right itself once placed on its back). hSOD1G93A × TLR4−/− shows a significant extension in survival time relative to hSOD1G93A mice (n = 11, P < 0.05, log-rank test). (B) shows hind-limb grip strength in these two groups. A small overall improvement in grip strength was seen in hSOD1G93A × TLR4−/− mice versus hSOD1G93A mice, with significant differences observed at 56, 63, 84 and 161 days of age (n = 8–9, *P < 0.05, +P < 0.01, Student’s t-test). Data are expressed as mean ± SEM.