Thus, our present findings of the interactions between DCTN1and TDP-43, as well as DCTN1 regulation of TDP-43 aggregation, have opened a new avenue toward elucidating not only the physiological mechanisms by which the dynein–dynactin machinery controls TDP-43 transport and distribution but also the pathological mechanisms underlying Perry disease and other TDP-43 proteinopathies, including ALS/FTD. Here, DCTN1 is linked to amyotrophic lateral sclerosis.