More specifically for C9‐HRE‐associated ALS, mouse models expressing DPRs or reduced levels of C9ORF72 demonstrated microglial activation and onset of neuroinflammation (O'Rourke et al. 2016; Zhou et al. 2020; Zhu et al. 2020). Here, C9orf72 is linked to amyotrophic lateral sclerosis.