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. 2009 Feb 27;106(11):4465–4470. doi: 10.1073/pnas.0813339106

Fig. 2.

Fig. 2.

Selective mutant SOD1 gene excision from Schwann cells accelerates disease progression in ALS mice. (A–C) Plots of ages (in days) at which disease onset (A), early disease (B), and end stage (C) are reached in LoxSOD1G37R ALS mice with (blue) and without (red) mutant SOD1 in Schwann cells. (D) Correlation between dismutase activity of Schwann cell-localized SOD1 mutants with slow disease progression in ALS mice: removal of dismutase active mutant SOD1G37R from Schwann cells leads to a 3-fold faster late phase (from early disease to end stage) in LoxSOD1G37R/P0-cre mice than in LoxSOD1G37R mice (P < 0.01; Student's t test). This difference correlates well with a faster late phase in dismutase inactive mutant SOD1G85R mice (line 148; end stage at 12.5 months) as compared to a slow late phase in dismutase active mutant SOD1G37R mice (line 106; end stage at 13.5 months) (P < 0.001; Student's t test) (see Discussion).