Since then, an increasing number of genes have been identified for which the splicing of annotated, conserved exons is regulated by TDP-43; as such, altered splicing in POLDIP3, SORT1, and PFKP have all been used as reliable readouts for nuclear TDP-43 function in models with TDP-43 knockout, overexpression, and for studying TDP-43 ALS-causing mutations [17–27]. Here, TARDBP is linked to amyotrophic lateral sclerosis.