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Figure 2 | Orphanet Journal of Rare Diseases

Figure 2

From: VPAC2 receptor agonist BAY 55-9837 increases SMN protein levels and moderates disease phenotype in severe spinal muscular atrophy mouse models

Figure 2

BAY 55-9837 treatment increases SMN expression via p38 MAPK pathway. (a) Representative western blot showing activation of p38 MAPK pathway upon BAY 55-9837 treatment in NT2 cells. NT2 cells were treated with BAY 55-9837 at indicated times and then harvested for western blot analysis. Activation of p38 pathway by BAY 55-9837 leads to an increase in SMN protein. (b) Densitometric quantification of phospho-p38 relative to total-p38 (the ratio at control treatment was set as 1; mean + SEM (bars) of three independent experiments) are shown for NT2 cells. (c) 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 NT2 cells. (d) Representative western blots showing the effect of p38 inhibition on BAY 55-9837-induced increase in SMN protein. p38 inhibitor (SB-239580) blocked the BAY 55-9837-induced increase in SMN protein in NT2 cells. NT2 cells were treated with SB-239580 (p38 In; 3 μM) for 2 h followed by treatment with BAY 55-9837 for 24 h and than harvested for western blot analysis. (e) 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 NT2 cells showing the effect of p38 inhibition on BAY 55-9837-induced increase in SMN protein. *P < 0.05; **P < 0.01, t-test.

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