Figure 1. The effects of butyrate (BA) and 4-phenylbutyrate (4PBA) on survival of SMNΔ7 SMA mice.
SMNΔ7 SMA mice were treated daily with BA (A-B) or 4PBA (C-D) starting at PND04 and monitored for changes in lifespan (A, C) and onset of loss of body mass (B, D). Daily oral administration of BA (5 g/kg/d, t.i.d.) had no effect on the survival (A; p = 0.253) or the onset of body mass loss (B; p = 0.925) in SMNΔ7 SMA mice. The BA analogue 4PBA (500 mg/kg/d, t.i.d.) increased the average lifespan of SMNΔ7 SMA mice by 53% (C; p = 0.026) but not significantly delay the onset of loss of body mass (D; p = 0.190).