FUS and amyotrophic lateral sclerosis: In addition to changes in stress granule dynamics, mouse models of ALS also demonstrated that mislocalisation of RBPs and changes in their splicing activities are sufficient to cause motor neuron degeneration, without nuclear depletion and cytoplasmic aggregation, such as in the humanised mouse model of FUS-ALS [132] and a TDP-43 gain-of-function ALS model [133].