Mislocalization and accumulation of hyperphosphorylated, fragmented, and ubiquitinated forms of this protein in the cytoplasm of neurons are known as TDP43 proteinopathy, a pathological hallmark of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) (2, 3). Here, TARDBP is linked to proteostasis deficiencies.