Besides, mice models overexpressing ALS-linked TDP-43 mutants, such as Q331K [66, 81–83], are likely to involve specific dysregulation in RNA splicing complex formation due to perturbed binding of hnRNP factors – hnRNPA1, hnRNPA2/B1, E2 [39, 84, 85] at the C-terminal prion-like domain of TDP-43, instead of general TDP-43 aggregation pathobiology. Here, TARDBP is linked to amyotrophic lateral sclerosis.