TDP-43 pathology is associated with activation of ISR in both neurons and non-neuron cells in nervous system postmortem tissues of people who have died with ALS or FTD [41], with increased CHOP levels identified in multiple types of cells in the postmortem tissues of human ALS [17] as well as in cell and mouse models for ALS and FTD [15, 20, 42, 43]. Here, TARDBP is linked to amyotrophic lateral sclerosis.