Mislocalization of transactive response DNA binding protein of 43 kDa (TDP-43) is a pathological hallmark of frontotemporal dementia and amyotrophic lateral sclerosis (FTD/ALS), two related neurodegenerative diseases, and is a co-pathology in up to 57% of Alzheimer’s disease cases1. Here, TARDBP is linked to amyotrophic lateral sclerosis.