ANK3 expression was also downregulated in postmortem ALS spinal cords (Figure S1C) and was associated with a reduction in AIS length measured by ankyrin-G staining (Figures S1E–S1G), in line with previously published data.38 It is likely that onset of TDP-43 mis-localization in the later neurons partly contributes to this switch in AIS length through changes in gene expression. Here, TARDBP is linked to amyotrophic lateral sclerosis.