TDP-43 is a major component of neuronal inclusions characteristic of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar dementia (FTLD), and many other neurodegenerative diseases collectively called TDP-43 proteinopathies (Neumann et al., 2006; Gao et al., 2017; Nelson et al., 2019; Tziortzouda et al., 2021). Here, TARDBP is linked to proteostasis deficiencies.