TARDBP and amyotrophic lateral sclerosis: Of note, the reported heterozygous ALS-associated TDP-43p.Y374Ter truncation variant [13, 14, 30] scores lower than WT TDP-43 in the same prion prediction in silico tools (PAPA-score of 0.036) (data not shown), further suggesting an important role of the novel 9 aa.