Similarly, bifunctional TOES, whose tail of enhancer sequences recruits activating proteins such as positively acting SR proteins, has been used to increase the splicing of refractory exon 7 in SMN2 in fibroblasts derived from patients with SMA (Skordis et al., 2003; Owen et al., 2011). This evidence concerns the gene SMN2 and proximal spinal muscular atrophy.