TARDBP and amyotrophic lateral sclerosis: Since mVCP fibroblasts successfully demonstrated the rescue of pathological features in human cells by upregulation of the HSR, we began our study in human induced pluripotent stem cell (iPSC)-derived motor neurons (iPSC-MNs) established from ALS patients expressing mutant VCP, which provide a more complex, neuronal and highly disease-specific cell culture model of neurodegeneration, and which have been previously shown to manifest a TDP-43 pathology [53].