Figure 1

BAY 55-9837 treatment upregulates SMN protein in vitro . NT2, MN-1 and SMA I patient fibroblasts were treated with BAY 55-9837 (0.25 μM) and then harvested at 24 hours for western blot analyses. (a) Representative western blots showing the effect of BAY 55-9837 on SMN protein in NT2 cells. (b) Densitometric quantification of SMN protein relative to Actin (the ratio at control treatment was set as 1; mean + SEM (bars) of six independent experiments) are shown for NT2 cells. (c) Representative western blot showing effect of BAY 55-9837 on SMN protein in MN-1 cells. (d) Densitometric quantification of SMN protein relative to Tubulin (the ratio at control treatment was set as 1; mean + SEM (bars) of three independent experiments) are shown for MN-1 cells. (e) Representative western blots showing the effect of BAY 55-9837 on SMN protein in SMA I patient fibroblasts (all lanes were run on the same gel but were non-contiguous). (f) Densitometric quantification of SMN protein relative to Tubulin (the ratio at control treatment was set as 1; mean + SEM (bars) of five independent experiments) are shown for SMA I patient fibroblasts. *P < 0.05; **P < 0.01; ***P < 0.001, t-test.