Given the strong evidence of programmed cell death and inflammation occurring in SMA, we generated a mouse model to study the pathway by knocking out Ripk3 and Casp1. RIPK3 is a crucial mediator of necroptosis and homozygous deletion of Ripk3 should target the necroptosis pathway (Zhang et al., 2011; Rodriguez et al., 2016). Here, RIPK3 is linked to proximal spinal muscular atrophy.