The change of the conformational state of TDP-43 by mutations in its primary structure or the introduction of modifications and the formation of ß- and amyloid-like structures, together with evidence for oligomerization and cell-to-cell transmission, implies a disease-specific readout known from other neurodegenerative diseases [22, 45, 67, 93, 94]. Here, TARDBP is linked to neurodegenerative disease.