TARDBP and amyotrophic lateral sclerosis: Besides the aberrant aggregation of transactive response DNA-binding protein of 43 kDa (TDP-43) (5, 70), the nuclear-to-cytoplasmic mislocalization of fused in sarcoma (FUS) protein in motor neurons has been identified as another pathogenic feature of VCP-mutated ALS, which was ascribed to the increased intron retention (IR) in splicing factor proline and glutamine rich (SFPQ) transcripts (71, 72).