Our hypothesis that dysregulation of DCTN1–TDP-43 interactions and/or the imbalance in DCTN1 expression perturb TDP-43 transport from axonal tips to the nucleus, and cause TDP-43 aggregation in the cytoplasm and axoplasm may also be applicable to ALS or ALS-related neurodegenerative diseases. Here, DCTN1 is linked to amyotrophic lateral sclerosis.