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. 2006 Apr 24;103(18):7142–7147. doi: 10.1073/pnas.0602046103

Fig. 1.

Fig. 1.

Kaplan–Meier plots showing the age of onset and cumulative survival of the single- and double-transgenic mice. (Top) hwtSOD1 exacerbates the disease in SOD1G93A (shown as G93A) transgenic mice. The G93A/hwtSOD1 double-transgenic mice (red line; n = 21) have an earlier onset of disease (76.8 ± 8.7 days vs. 103.2 ± 9.9 days; log-rank test χ2 = 97.63, P < 0.0001) (Left) and a shorter lifespan (117.7 ± 7.7 days vs. 127.4 ± 7.6 days; log-rank test χ2 = 20.81, P < 0.0001) (Right) than the G93A single-transgenic mice (blue line; n = 58, sibling control). (Middle) hwtSOD1 converts an unaffected phenotype of a SOD1A4V (shown as A4V) transgenic mouse line to an ALS-like phenotype. The A4V transgenic mice do not develop disease in their lifetime (>600 days; n = 23). However, the A4V/hwtSOD1 double-transgenic mice (n = 6) de novo develop ALS-like disease (227.7 ± 50.7 days; χ2 = 41.91, P < 0.0001) (Left) with a shorter lifespan (318.5 ± 41.7 days; χ2 = 41.01, P < 0.0001) (Right). (Bottom) hwtSOD1 exacerbates the disease in SOD1L126Z (shown as L126Z) transgenic mice. The L126Z/hwtSOD1 double-transgenic mice have a much earlier onset of disease [178.3 ± 19.1 days (n = 7) vs. 336.3 ± 42.7 days (n = 18); χ2 = 34.38, P < 0.0001] (Left) and a much shorter lifespan than the single-transgenic mice (201.4 ± 14.5 days vs. 359 ± 41.1 days; χ2 = 34.38, P < 0.0001) (Right).