As SMN is a ubiquitously expressed protein and metabolic dysregulations associated with SMA have been reported [123,124,125], heterozygous Smn-depleted [126] and severe SMA (Smn−/−; SMN2+/0) [127] mouse models had been employed to investigate the role of SMN in metabolism. This evidence concerns the gene SMN1 and proximal spinal muscular atrophy.