Finally, partial impairment of TBK1 activity via an ALS-associated R228H point mutation, combined with (GA)100 peptide expression (C9ORF72 dipeptide mimic), showed increased TDP-43 pathology and defects in endolysosomal flux (Shao et al., 2022). Here, TARDBP is linked to amyotrophic lateral sclerosis.