Fig. 9

Selective up-regulation of transcripts involved in cell death in vulnerable motor neurons. a Gene list show transcripts associated with cell death which were statistically significantly altered in SMAv compared to WTv motor neurons. Arrow indicates transcripts that were investigated by qPCR. b Bar charts (Mean ± SEM) showing relative expression of PMAIP and CDKN1a in WTv, SMAv, WTr and SMAr motor neurons. Note that qPCR results confirmed up-regulation of PMAIP and CDKN1a in SMAv motor neurons compared to WTv. There was also a significant up-regulation of CDKN1a in SMAr motor neurons. N = 4 biological replicates, each approximately 200 motor neurons from 2–3 mice. c Bar charts (Mean ± SEM) showing relative expression of PMAIP, Fas and CDKN1a in whole spinal cord from Smn ^2B/- and Smn ^−/− ;SMN2 mice compared to wild-type or Smn ^+/+ ;SMN2 (control) respectively. This showed an up-regulation of all transcripts in SMA mouse models, with the exception of CDKN1a in Smn ^−/− ;SMN2 samples. Note that this demonstrates the up-regulation of transcripts associated with cell death pathways can be seen at pre-symptomatic time points in two different mouse models of SMA. ***P < 0.001, *P < 0.05 by Mann Whitney U test