In line with previous studies on the association between TMEM106B genetic variants and TDP-43 aggregation pathology [53, 90], our data provide new evidence that TMEM106B could be related to the nuclear clearance of TDP-43 specifically in C9-ALS and C9-ALS/FTD at the cellular level. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.