In agreement with these findings, lowering MMP9 levels or its genetic removal was shown to extend the survival of MNs (Kaplan et al. 2014; Kiaei et al. 2007) with protective effects in SOD1 and TDP-43 ALS mice (Lorenzl et al. 2006; Kaplan et al. 2014; Spiller et al. 2019). Here, TARDBP is linked to amyotrophic lateral sclerosis.