SMN directly interacts with TRP53 (Young et al. 2002), and transcriptional activation of Trp53 or its target genes has been previously shown in different models of SMA (Zhang et al. 2008, 2013; Bäumer et al. 2009; Murray et al. 2015; Staropoli et al. 2015; Jangi et al. 2017; Simon et al. 2017). Here, SMN1 is linked to proximal spinal muscular atrophy.