Fig 5.
SMN deficiency impairs exon 7 splicing in SMA motor neurons. Analysis of the relative efficiency of exon 7 inclusion in the whole spinal cord (A) as well as motor neurons (LCM MNs) and non-motor neurons (LCM non-MNs) (B) isolated from control (Smn+/−; SMN2+/+; SMNΔ7+/+; GFP+/−) and Δ7 SMA (Smn−/−; SMN2+/+; SMNΔ7+/+; GFP+/−) mice at postnatal day 6. Normalized exon 7 inclusion was measured by real-time RT-qPCR analysis of the expression of full-length (SMN2FL) relative to total (SMN2TOT) SMN2 mRNAs. Values represent means and SEM from independent experiments (n=8; *, P < 0.05).
