SMN2 and proximal spinal muscular atrophy: Kannan et al. also show that the upregulation of ZPR1 in SMN mutated mice exhibited significantly higher levels of SETX, increased levels of DNA-PKcs, and a lower number of R-loops, thereby ameliorating the severity of the SMA phenotype (Kannan et al., 2018, Kannan et al., 2020).