Fig. 7.

TDP-43 promotes the levels of the targets of LEC and SEC contributing to neurodegeneration. A model for TDP-43 toxicity associated with LEC and SEC. LEC regulates the transcription of snRNAs, forming spliceosomes, and SEC regulates stress response genes, maintaining cell homeostasis. In the disease state, TDP-43 promotes the levels of LEC and SEC targets through interactions with Ell in fly/ELL2 in human cells, including U12 and Hsrω/Sat III, leading to misregulation of splicing, cell loss, and disruption of cell homeostasis and contributes to neurodegeneration