USP13 was identified as a downstream effector of TDP-43, a protein implicated in several neurodegenerative disorders including amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), and Alzheimer’s disease (AD) (Chen and Mitchell, 2021; Josephs et al., 2014; Neumann et al., 2006). Here, USP13 is linked to frontotemporal dementia.