Although the reasons why a decreased availability of SMN activates a very selective process that leads to the specific degeneration of lower motor neurons is far from being clear, evidence exist that mys-functioning in SMN-regulated processes might be shared by ALS (Yamazaki et al., 2012; Gerbino et al., 2013; Groen et al., 2013; Turner et al., 2014), further indicating that motor neurons are particularly vulnerable to disturbed RNA metabolism. This evidence concerns the gene SMN1 and amyotrophic lateral sclerosis.