The aggregation of ALS-associated SOD1 – an antioxidant enzyme highly mutated in more than 20% of familial ALS cases (Corson et al., 1998; Gruzman et al., 2007; Berdyński et al., 2022) and TDP-43–a protein involved in RNA processing regulation (Hergesheimer et al., 2019) occurs in motor neuron axons and results in autophagy dysregulation (Sasaki, 2011; Wei, 2014; Budini et al., 2017). Here, TARDBP is linked to amyotrophic lateral sclerosis.