Since there is mounting evidence that p53 plays a role in motor neuron death in different SMA mouse models (Simon et al., 2017; Courtney et al., 2019) and that p53 mediates neuronal cell death by necroptosis (Peek et al., 2022), it is important to investigate the contribution of necroptosis to motor neuron and muscle pathology in SMA. Here, TP53 is linked to proximal spinal muscular atrophy.