SMN2 and proximal spinal muscular atrophy: Moreover, Olesoxime showed neuroprotective and neuroregenerative effects in several animal models of motor nerve degeneration and in a transgenic mouse model of severe SMA (SmnF7/F7; NSE-Cre mice; mutant mice carrying a deletion of Smn exon seven directed to neurons): daily administrations of Olesoxime extended the survival of the treated mice (Bordet et al., 2010).