Importantly, p53 appears to play a key role in C9ORF72-ALS and DPR-induced toxicity as p53 knockout or knockdown has been shown to extend the lifespan of a mouse model expressing poly(PR), and protect against neurodegeneration in Drosophila models expressing the C9ORF72 repeat expansion [74]. Here, TP53 is linked to amyotrophic lateral sclerosis.