TDP-43 pathology is described in a growing number of diseases collectively known as TDP-43 proteinopathies, including ALS, ∼45% of FTD cases, and a recently described subtype of old-age dementia called limbic-predominant age-related TDP-43 encephalopathy (LATE) (de Boer et al., 2020; Hardiman et al., 2017; Nelson et al., 2019). The gene discussed is TARDBP; the disease is proteostasis deficiencies.